Neonatal Magnetic Resonance Imaging Pattern of Brain Injury as a Biomarker of Childhood Outcomes following a Trial of Hypothermia for Neonatal Hypoxic-Ischemic Encephalopathy JOURNAL OF PEDIATRICS 2015; 167 (5): 987-?
Intensity-Corrected Dual-Echo Echo-Planar Imaging (DE-EPI) for Improved Pediatric Brain Diffusion Imaging PLOS ONE 2015; 10 (6)
The Significance of Macrocephaly or Enlarging Head Circumference in Infants With the Triad Further Evidence of Mimics of Shaken Baby Syndrome AMERICAN JOURNAL OF FORENSIC MEDICINE AND PATHOLOGY 2015; 36 (2): 111-120
Infants with the triad (neurologic dysfunction, subdural hematoma [SDH], and retinal hemorrhage) are often diagnosed as victims of shaken baby syndrome. Medical conditions/predisposing factors to developing the triad are often dismissed: short falls, birth-related SDH that enlarges, macrocephaly, sinus/cortical vein thrombosis, and others. Six infants with the triad are described in which child abuse was diagnosed, but parents denied wrongdoing. All 6 had either macrocephaly or enlarging head circumference, which suggested medical explanations. Three infants incurred short falls, 1 had a difficult delivery in which there was likely a rebleed of a birth-related SDH, 1 had a spontaneous SDH associated with increased extra-axial fluid spaces, and 1 had a sinus thrombosis. Following legal proceedings, all 6 infants were returned to their parents, and there has been no child maltreatment in follow-up, suggesting child abuse never happened. The results indicate that alternative medical explanations for causing the triad should be considered and that macrocephaly or an enlarging head circumference raises the possibility of a medical explanation.
View details for DOI 10.1097/PAF.0000000000000152
View details for Web of Science ID 000354388100015
View details for PubMedID 25893912
Decreased tumor apparent diffusion coefficient correlates with objective response of pediatric low-grade glioma to bevacizumab JOURNAL OF NEURO-ONCOLOGY 2015; 122 (3): 491-496
Recent small, retrospective series suggest bevacizumab may be a therapeutic option for recurrent pediatric low-grade glioma (LGG). Assessment of therapeutic responses is complicated by the unpredictable natural history of these tumors. Because diffusion-weighted imaging quantifies microscopic water motion affected by cellular density and histologic features, we hypothesized that it may be helpful in monitoring therapeutic response of LGG to bevacizumab. We retrospectively reviewed eight consecutive patients, median age 4.8 (range 2.3-12.3) years at initiation of bevacizumab therapy for recurrent or refractory LGG. Patients received 10 mg/kg/dose every 2 weeks (median 16 doses/therapy course). Mean apparent diffusion coefficient (ADC) was measured and analyzed in respect to tumor volume. Following the first treatment course, seven of eight patients had reduced tumor volume (25 %) and ADC. The median decrease in tumor volume was 47% (range -6 to 78 %) and the median decrease in ADC was 14 % (range -5 to 30 %). The ADC was significantly decreased during therapy, whereas the decrease in volume was seen only after therapy completion. There was a positive correlation between percent change in tumor volume and ADC (p < 0.05). We report a decrease in tumor ADC during initial bevacizumab therapy that is accompanied by a decrease in volume following therapy. Imaging changes in microscopic water motion associated with histology may be useful in monitoring the therapeutic response of LGG to bevacizumab.
View details for DOI 10.1007/s11060-015-1754-9
View details for Web of Science ID 000354717800008
Effect of Number of Acquisitions in Diffusion Tensor Imaging of the Pediatric Brain: Optimizing Scan Time and Diagnostic Experience JOURNAL OF NEUROIMAGING 2015; 25 (2): 296-302
Diffusion tensor imaging (DTI) is useful for multiple clinical applications, but its routine implementation for children may be difficult due to long scan times. This study evaluates the impact of decreasing the number of DTI acquisitions (NEX) on interpretability of pediatric brain DTI.15 children with MRI-visible neuropathologies were imaged at 3T using our motion-corrected, parallel imaging- accelerated DT-EPI technique with 3 NEX (scan time 8.25 min). Using these acquisitions, NEX = 1 (scan time 2.75 min) and NEX = 2 (scan time 5.5 min) images were simulated. Two neuroradiologists scored diffusion-weighted images (DWI), apparent diffusion coefficient (ADC), fractional anisotropy (FA), and first eigenvector color-encoded (EV) images from each NEX for perceived SNR, lesion conspicuity and clinical confidence. ROI FA/ADC and image SNR values were also compared across NEX.NEX = 2 perceived SNR, lesion conspicuity, and clinical confidence were not inferior to NEX = 3 images. NEX = 1 images showed comparable lesion conspicuity and clinical confidence as NEX = 3, but inferior perceived SNR. FA and ADC ROI measurements demonstrated no significant difference across NEX. The greatest SNR increase was seen between NEX = 1 and NEX = 2.Reducing NEX to shorten imaging time may impact clinical utility in a manner that does not directly correspond with SNR changes.
View details for DOI 10.1111/jon.12093
View details for Web of Science ID 000351306000020
Neuroimaging and neurodevelopmental outcome in extremely preterm infants. Pediatrics 2015; 135 (1): e32-42
Extremely preterm infants are at risk for neurodevelopmental impairment (NDI). Early cranial ultrasound (CUS) is usual practice, but near-term brain MRI has been reported to better predict outcomes. We prospectively evaluated MRI white matter abnormality (WMA) and cerebellar lesions, and serial CUS adverse findings as predictors of outcomes at 18 to 22 months' corrected age.Early and late CUS, and brain MRI were read by masked central readers, in a large cohort (n = 480) of infants <28 weeks' gestation surviving to near term in the Neonatal Research Network. Outcomes included NDI or death after neuroimaging, and significant gross motor impairment or death, with NDI defined as cognitive composite score <70, significant gross motor impairment, and severe hearing or visual impairment. Multivariable models evaluated the relative predictive value of neuroimaging while controlling for other factors.Of 480 infants, 15 died and 20 were lost. Increasing severity of WMA and significant cerebellar lesions on MRI were associated with adverse outcomes. Cerebellar lesions were rarely identified by CUS. In full multivariable models, both late CUS and MRI, but not early CUS, remained independently associated with NDI or death (MRI cerebellar lesions: odds ratio, 3.0 [95% confidence interval: 1.3-6.8]; late CUS: odds ratio, 9.8 [95% confidence interval: 2.8-35]), and significant gross motor impairment or death. In models that did not include late CUS, MRI moderate-severe WMA was independently associated with adverse outcomes.Both late CUS and near-term MRI abnormalities were associated with outcomes, independent of early CUS and other factors, underscoring the relative prognostic value of near-term neuroimaging.
View details for DOI 10.1542/peds.2014-0898
View details for PubMedID 25554820
Neuroimaging and Neurodevelopmental Outcome in Extremely Preterm Infants PEDIATRICS 2015; 135 (1): E32-E42
Intensity-Corrected Dual-Echo Echo-Planar Imaging (DE-EPI) for Improved Pediatric Brain Diffusion Imaging. PloS one 2015; 10 (6)
Here we investigate the utility of a dual-echo Echo-Planar Imaging (DE-EPI) Diffusion Weighted Imaging (DWI) approach to improve lesion conspicuity in pediatric imaging. This method delivers two 'echo images' for one diffusion-preparation period. We also demonstrate how the echoes can be utilized to remove transmit/receive coil-induced and static magnetic field intensity modulations on both echo images, which often mimic pathology and thereby pose diagnostic challenges. DE-EPI DWI data were acquired in 18 pediatric patients with abnormal diffusion lesions, and 46 pediatric patient controls at 3T. Echo1 [TE = 45ms] and Echo2 [TE = 86ms] were corrected for signal intensity variation across the images by exploiting the images equivalent coil-sensitivity and susceptibility-induced modulations. Two neuroradiologists independently reviewed Echo1 and Echo2 and their intensity-corrected variants (cEcho1 and cEcho2) on a 7-point Likert scale, with grading on lesion conspicuity diagnostic confidence. The apparent diffusion coefficient (ADC) map from Echo1 was used to validate presence of true pathology. Echo2 was unanimously favored over Echo1 for its sensitivity for detecting acute brain injury, with a mean respective lesion conspicuity of 5.7/4.4 (p < 0.005) and diagnostic confidence of 5.1/4.3 (p = 0.025). cEcho2 was rated higher than cEcho1, with a mean respective lesion conspicuity of 5.5/4.3 (p < 0.005) and diagnostic confidence of 5.4/4.4 (p < 0.005). cEcho2 was favored over all echoes for its diagnostic reliability, particularly in regions close to the head coil. This work concludes that DE-EPI DWI is a useful alternative to conventional single-echo EPI DWI, whereby Echo2 and cEcho2 allows for improved lesion detection and overall higher diagnostic confidence.
View details for DOI 10.1371/journal.pone.0129325
View details for PubMedID 26069959
Diffusion-weighted MRI derived apparent diffusion coefficient identifies prognostically distinct subgroups of pediatric diffuse intrinsic pontine glioma JOURNAL OF NEURO-ONCOLOGY 2014; 117 (1): 175-182
While pediatric diffuse intrinsic pontine gliomas (DIPG) remain fatal, recent data have shown subgroups with distinct molecular biology and clinical behavior. We hypothesized that diffusion-weighted MRI can be used as a prognostic marker to stratify DIPG subsets with distinct clinical behavior. Apparent diffusion coefficient (ADC) values derived from diffusion-weighted MRI were computed in 20 consecutive children with treatment-nave DIPG tumors. The median ADC for the cohort was used to stratify the tumors into low and high ADC groups. Survival, gender, therapy, and potential steroid effects were compared between the ADC groups. Median age at diagnosis was 6.6 (range 2.3-13.2) years, with median follow-up seven (range 1-36) months. There were 14 boys and six girls. Seventeen patients received radiotherapy, five received chemotherapy, and six underwent cerebrospinal fluid diversion. The median ADC of 1,29510(-6)mm(2)/s for the cohort partitioned tumors into low or high diffusion groups, which had distinct median survivals of 3 and 13months, respectively (log-rank p<0.001). Low ADC tumors were found only in boys, whereas high ADC tumors were found in both boys and girls. Available tissue specimens in three low ADC tumors demonstrated high-grade histology, whereas one high ADC tumor demonstrated low-grade histology with a histone H3.1 K27M mutation and high-grade metastatic lesion at autopsy. ADC derived from diffusion-weighted MRI may identify prognostically distinct subgroups of pediatric DIPG.
View details for DOI 10.1007/s11060-014-1375-8
View details for Web of Science ID 000331964600021
Arterial Spin-Labeled Perfusion of Pediatric Brain Tumors AMERICAN JOURNAL OF NEURORADIOLOGY 2014; 35 (2): 395-401
Pediatric brain tumors have diverse pathologic features, which poses diagnostic challenges. Although perfusion evaluation of adult tumors is well established, hemodynamic properties are not well characterized in children. Our goal was to apply arterial spin-labeling perfusion for various pathologic types of pediatric brain tumors and evaluate the role of arterial spin-labeling in the prediction of tumor grade.Arterial spin-labeling perfusion of 54 children (mean age, 7.5 years; 33 boys and 21 girls) with treatment-naive brain tumors was retrospectively evaluated. The 3D pseudocontinuous spin-echo arterial spin-labeling technique was acquired at 3T MR imaging. Maximal relative tumor blood flow was obtained by use of the ROI method and was compared with tumor histologic features and grade.Tumors consisted of astrocytic (20), embryonal (11), ependymal (3), mixed neuronal-glial (8), choroid plexus (5), craniopharyngioma (4), and other pathologic types (3). The maximal relative tumor blood flow of high-grade tumors (grades III and IV) was significantly higher than that of low-grade tumors (grades I and II) (P < .001). There was a wider relative tumor blood flow range among high-grade tumors (2.14 1.78) compared with low-grade tumors (0.60 0.29) (P < .001). Across the cohort, relative tumor blood flow did not distinguish individual histology; however, among posterior fossa tumors, relative tumor blood flow was significantly higher for medulloblastoma compared with pilocytic astrocytoma (P = .014).Characteristic arterial spin-labeling perfusion patterns were seen among diverse pathologic types of brain tumors in children. Arterial spin-labeling perfusion can be used to distinguish high-grade and low-grade tumors.
View details for DOI 10.3174/ajnr.A3670
View details for Web of Science ID 000331279300035
Improved T2*Imaging without Increase in Scan Time: SWI Processing of 2D Gradient Echo AMERICAN JOURNAL OF NEURORADIOLOGY 2013; 34 (11): 2092-2097
BACKGROUND AND PURPOSE:2D gradient-echo imaging is sensitive to T2* lesions (hemorrhages, mineralization, and vascular lesions), and susceptibility-weighted imaging is even more sensitive, but at the cost of additional scan time (SWI: 5-10 minutes; 2D gradient-echo: 2 minutes). The long acquisition time of SWI may pose challenges in motion-prone patients. We hypothesized that 2D SWI/phase unwrapped images processed from 2D gradient-echo imaging could improve T2* lesion detection.MATERIALS AND METHODS:2D gradient-echo brain images of 50 consecutive pediatric patients (mean age, 8 years) acquired at 3T were retrospectively processed to generate 2D SWI/phase unwrapped images. The 2D gradient-echo and 2D SWI/phase unwrapped images were compared for various imaging parameters and were scored in a blinded fashion.RESULTS:Of 50 patients, 2D gradient-echo imaging detected T2* lesions in 29 patients and had normal findings in 21 patients. 2D SWI was more sensitive than standard 2D gradient-echo imaging in detecting T2* lesions (P < .0001). 2D SWI/phase unwrapped imaging also improved delineation of normal venous structures and nonpathologic calcifications and helped distinguish calcifications from hemorrhage. A few pitfalls of 2D SWI/phase unwrapped imaging were noted, including worsened motion and dental artifacts and challenges in detecting T2* lesions adjacent to calvaria or robust deoxygenated veins.CONCLUSIONS:2D SWI and associated phase unwrapped images processed from standard 2D gradient-echo images were more sensitive in detecting T2* lesions and delineating normal venous structures and nonpathologic mineralization, and they also helped distinguish calcification at no additional scan time. SWI processing of 2D gradient-echo images may be a useful adjunct in cases in which longer scan times of 3D SWI are difficult to implement.
View details for DOI 10.3174/ajnr.A3595
View details for Web of Science ID 000330234700008
Increased focal hemosiderin deposition in pediatric medulloblastoma patients receiving radiotherapy at a later age. Journal of neurosurgery. Pediatrics 2013; 12 (5): 444-451
Object Focal hemosiderin deposition (FHD) is commonly observed on brain MRI scans of patients treated for childhood medulloblastoma (MB). The authors sought to determine the clinical significance of FHD and its relationship to patient age, radiation dose, and cognitive outcomes. Methods A single-institution retrospective study of 93 MB patients at Lucile Packard Children's Hospital at Stanford from 1998 to 2011 identified 41 patients with a negative baseline MRI scan and at least 2 posttreatment MRI scans obtained with T2* gradient recalled echo (GRE). The number and cumulative rate of FHDs detectable by GRE were compared between patients aged 6 years and younger (early age) and aged 7-21 years (late age) at the time of radiotherapy (RT) and between low-dose (1800-2340 cGy) and high-dose (2920-3960 cGy) RT. Results The median age at MB diagnosis was 7.3 years (range 0.9-21.0 years), the median clinical follow-up period was 5.8 years (range 0.8-13.4 years), and the median 5-year overall survival was 81% 7%. Of 30 school-aged children with MB, 21 (70%) required special education, and the median IQ of 10 tested patients was 100 (range 50-118). Thirty-three patients (80%) had FHD after a median latency of 1.9 years (range 0.1-5.9 years). Ninety-four percent (436 of 466) of the lesions arose in the supratentorial region of the brain, whereas 29 (6%) resided in the brainstem or the cerebellum. No spinal lesions were observed on routine spine MRI scans using T2 fast spin echo imaging. The mean cumulative lesion rate per year was 2.23 3.05, and this rate was higher in older children at the time of RT compared with younger children (3.23 vs 0.67 per year, p = 0.002) but did not differ among different RT doses (p = 0.395). A child's IQ or need for special education showed no significant correlation with the rate of lesion development or number of lesions. None of the lesions resulted in symptomatic hemorrhage that required surgical intervention. Conclusions More FHD was observed in children treated for MB at the older ages than in those treated at the younger ages. There was no significant association of the incidence of FHD with radiation dose or cognitive outcomes, and none of the lesions required surgical intervention.
View details for DOI 10.3171/2013.7.PEDS1330
View details for PubMedID 23992236
Reduced Cerebral Arterial Spin-Labeled Perfusion in Children with Neurofibromatosis Type 1 AMERICAN JOURNAL OF NEURORADIOLOGY 2013; 34 (9): 1823-1828
BACKGROUND AND PURPOSE:Neurofibromatosis type 1 is associated with increased risk for stroke, cerebral vasculopathy, and neurocognitive deficits, but underlying hemodynamic changes in asymptomatic children remain poorly understood. We hypothesized that children with neurofibromatosis type 1 have decreased cerebral blood flow.MATERIALS AND METHODS:Arterial spin-labeled CBF was measured in 14 children with neurofibromatosis type 1 (median age, 9.7 years; mean, 10.2 years; range, 22 months to 18 years) and compared with age-matched control subjects on 3T MR imaging. Three-dimensional pseudocontinuous spin-echo arterial spin-labeled technique was used. Measurements were obtained at cortical gray matter of bilateral cerebral hemispheres and centrum semiovale by use of the ROI method. Comparison by Mann-Whitney test was used, with Bonferroni-adjusted P values .004 judged as significant.RESULTS:We identified 7 of 12 areas with significantly diminished arterial spin-labeled CBF in patients with neurofibromatosis type 1 compared with control subjects. These areas included the anterior cingulate gyrus (P = .001), medial frontal cortex (P = .004), centrum semiovale (P = .004), temporo-occipital cortex (P = .002), thalamus (P = .001), posterior cingulate gyrus (P = .002), and occipital cortex (P = .001). Among patients with neurofibromatosis type 1, there were no significant differences in these regions on the basis of the presence of neurofibromatosis type 1 spots or neurocognitive deficits.CONCLUSIONS:Reduced cerebral perfusion was seen in children with neurofibromatosis type 1, particularly in the posterior circulation and the vascular borderzones of the middle and posterior cerebral arteries.
View details for DOI 10.3174/ajnr.A3649
View details for Web of Science ID 000329848800034
View details for PubMedID 23764727
High-Risk Medulloblastoma: A Pediatric Oncology Group Randomized Trial of Chemotherapy Before or After Radiation Therapy (POG 9031) JOURNAL OF CLINICAL ONCOLOGY 2013; 31 (23): 2936-?
PURPOSETo compare event-free survival (EFS) in children with high-risk medulloblastoma randomly assigned to receive either chemotherapy before radiation or chemotherapy after radiation. PATIENTS AND METHODSOne hundred twelve patients were randomly assigned to each arm. Criteria used to categorize patients as high risk included M1-4 disease by modified Chang staging classification, T3b/T4 disease, or greater than 1.5 cm(3) of residual tumor after surgery. Postoperatively, children with high-risk medulloblastoma were randomly assigned to two arms, either chemotherapy entailing three cycles of cisplatin and etoposide before radiation (chemotherapy first [CT1]) or the same chemotherapy regimen after radiation (radiation therapy first [RT1]). Both groups received consolidation chemotherapy consisting of vincristine and cyclophosphamide.ResultsThe median follow-up time was 6.4 years. Five-year EFS was 66.0% in the CT1 arm and 70.0% in the RT1 arm (P = .54), and 5-year overall survival in the two groups was 73.1% and 76.1%, respectively (P = .47). In the CT1 arm, 40 of the 62 patients with residual disease achieved either complete or partial remission. CONCLUSIONFive-year EFS did not differ significantly whether, after surgery, patients received chemotherapy before or after radiotherapy.
View details for DOI 10.1200/JCO.2012.43.9984
View details for Web of Science ID 000330539300019
View details for PubMedID 23857975
Prognostic role for diffusion-weighted imaging of pediatric optic pathway glioma JOURNAL OF NEURO-ONCOLOGY 2013; 113 (3): 479-483
Optic pathway glioma (OPG) has an unpredictable course, with poor correlation between conventional imaging features and tumor progression. We investigated whether diffusion-weighted MRI (DWI) predicts the clinical behavior of these tumors. Twelve children with OPG (median age 2.7years; range 0.4-6.2years) were followed for a median 4.4years with DWI. Progression-free survival (time to requiring therapy) was compared between tumors stratified by apparent diffusion coefficient (ADC) from initial pre-treatment scans. Tumors with baseline ADC greater than 1,40010(-6)mm(2)/s required treatment earlier than those with lower ADC (log-rank p=0.002). In some cases, ADC increased leading up to treatment, and declined following treatment with surgery, chemotherapy, or radiation. Baseline ADC was higher in tumors that eventually required treatment (1,56219210(-6)mm(2)/s), compared with those conservatively managed (1,12311410(-6)mm(2)/s) (Kruskal-Wallis test p=0.013). Higher ADC predicted earlier tumor progression in this cohort and in some cases declined after therapy. Evaluation of OPG with DWI may therefore be useful for predicting tumor behavior and assessing treatment response.
View details for DOI 10.1007/s11060-013-1140-4
View details for Web of Science ID 000320461300017
Distinctive MRI Features of Pediatric Medulloblastoma Subtypes AMERICAN JOURNAL OF ROENTGENOLOGY 2013; 200 (4): 895-903
We hypothesized that the apparent diffusion coefficient (ADC) and other MRI features can be used to predict medulloblastoma histologic subtypes, as defined by the World Health Organization (WHO) in WHO Classification of Tumours of the Central Nervous System.A retrospective review of pediatric patients with medulloblastoma between 1989 and 2011 identified 38 patients with both pretreatment MRI and original pathology slides. The mean and minimum tumor ADC values and conventional MRI features were compared among medulloblastoma histologic subtypes.The cohort of 38 patients included the following histologic subtypes: 24 classic medulloblastomas, nine large cell (LC) or anaplastic medulloblastomas, four desmoplastic medulloblastomas, and one medulloblastoma with extensive nodularity. The median age at diagnosis was 8 years (range, 1-21 years) and the median follow-up time was 33 months (range, 0-150 months). The mean ADC ( 10(-3) mm(2)/s) was lower in classic medulloblastoma (0.733 0.046 [SD]) than in LC or anaplastic medulloblastoma (0.935 0.127) (Mann-Whitney test, p = 0.004). Similarly, the minimum ADC was lower in classic medulloblastoma (average SD, 0.464 0.056) than in LC or anaplastic medulloblastoma (0.630 0.053) (p = 0.004). The MRI finding of focal cysts correlated with the classic and desmoplastic subtypes (Fisher exact test, p = 0.026). Leptomeningeal enhancement positively correlated with the LC or anaplastic medulloblastoma subtype and inversely correlated with the classic medulloblastoma and desmoplastic medulloblastoma subtypes (p = 0.04). Ring enhancement correlated with tumor necrosis (p = 0.022) and with the LC or anaplastic medulloblastoma histologic subtype (p < 0.001).The LC or anaplastic medulloblastoma subtype was associated with increased ADC and with ring enhancement, the latter of which correlated with tumor necrosis. These features could be considered in the evaluation of high-risk medulloblastoma subtypes.
View details for DOI 10.2214/AJR.12.9249
View details for Web of Science ID 000316622100045
Brain injury following trial of hypothermia for neonatal hypoxic-ischaemic encephalopathy ARCHIVES OF DISEASE IN CHILDHOOD-FETAL AND NEONATAL EDITION 2012; 97 (6): F398-F404
The objective of our study was to examine the relationship between brain injury and outcome following neonatal hypoxic-ischaemic encephalopathy treated with hypothermia.Neonatal MRI scans were evaluated in the National Institute of Child Health and Human Development (NICHD) randomised controlled trial of whole-body hypothermia and each infant was categorised based upon the pattern of brain injury on the MRI findings. Brain injury patterns were assessed as a marker of death or disability at 18-22 months of age.Scans were obtained on 136 of 208 trial participants (65%); 73 in the hypothermia and 63 in the control group. Normal scans were noted in 38 of 73 infants (52%) in the hypothermia group and 22 of 63 infants (35%) in the control group. Infants in the hypothermia group had fewer areas of infarction (12%) compared to infants in the control group (22%). Fifty-one of the 136 infants died or had moderate or severe disability at 18 months. The brain injury pattern correlated with outcome of death or disability and with disability among survivors. Each point increase in the severity of the pattern of brain injury was independently associated with a twofold increase in the odds of death or disability.Fewer areas of infarction and a trend towards more normal scans were noted in brain MRI following whole-body hypothermia. Presence of the NICHD pattern of brain injury is a marker of death or moderate or severe disability at 18-22 months following hypothermia for neonatal encephalopathy.
View details for DOI 10.1136/archdischild-2011-301524
View details for Web of Science ID 000311022800003
View details for PubMedID 23080477
Diffusion tensor imaging (DTI) with retrospective motion correction for large-scale pediatric imaging JOURNAL OF MAGNETIC RESONANCE IMAGING 2012; 36 (4): 961-971
To develop and implement a clinical DTI technique suitable for the pediatric setting that retrospectively corrects for large motion without the need for rescanning and/or reacquisition strategies, and to deliver high-quality DTI images (both in the presence and absence of large motion) using procedures that reduce image noise and artifacts.We implemented an in-house built generalized autocalibrating partially parallel acquisitions (GRAPPA)-accelerated diffusion tensor (DT) echo-planar imaging (EPI) sequence at 1.5T and 3T on 1600 patients between 1 month and 18 years old. To reconstruct the data, we developed a fully automated tailored reconstruction software that selects the best GRAPPA and ghost calibration weights; does 3D rigid-body realignment with importance weighting; and employs phase correction and complex averaging to lower Rician noise and reduce phase artifacts. For select cases we investigated the use of an additional volume rejection criterion and b-matrix correction for large motion.The DTI image reconstruction procedures developed here were extremely robust in correcting for motion, failing on only three subjects, while providing the radiologists high-quality data for routine evaluation.This work suggests that, apart from the rare instance of continuous motion throughout the scan, high-quality DTI brain data can be acquired using our proposed integrated sequence and reconstruction that uses a retrospective approach to motion correction. In addition, we demonstrate a substantial improvement in overall image quality by combining phase correction with complex averaging, which reduces the Rician noise that biases noisy data.
View details for DOI 10.1002/jmri.23710
View details for Web of Science ID 000308884300022
View details for PubMedID 22689498
Newborn with prenatally diagnosed choroidal fissure cyst and panhypopituitarism and review of the literature. AJP reports 2011; 1 (2): 111-114
Little has been reported on fetal diagnosis of choroidal fissure cysts and prediction of the clinical complications that can result. We describe the case of a near-term male infant with prenatally diagnosed choroidal fissure cyst and bilateral clubfeet. His prolonged course in the neonatal intensive care nursery was marked by severe panhypopituitarism, late-onset diabetes insipidus, placement of a cystoperitoneal shunt, and episodes of sepsis. Postnatal genetic evaluation also revealed an interstitial deletion involving most of band 10q26.12 and the proximal half of band 10q26.13. The patient had multiple readmissions for medical and surgical indications and died at 6 months of age. This case represents the severe end of the spectrum of medical complications for children with choroidal fissure cysts. It highlights not only the importance of comprehensive evaluation and multidisciplinary management and counseling in such cases, but also the need for heightened vigilance in these patients.
View details for DOI 10.1055/s-0031-1293512
View details for PubMedID 23705098
Clinical Application of Readout-Segmented-Echo-Planar Imaging for Diffusion-Weighted Imaging in Pediatric Brain AMERICAN JOURNAL OF NEURORADIOLOGY 2011; 32 (7): 1274-1279
RS-EPI has been suggested as an alternative approach to EPI for high-resolution DWI with reduced distortions. To determine whether RS-EPI is a useful approach for routine clinical use, we implemented GRAPPA-accelerated RS-EPI DWI at our pediatric hospital and graded the images alongside standard accelerated (ASSET) EPI DWI used routinely for clinical studies.GRAPPA-accelerated RS-EPI DWIs and ASSET EPI DWIs were acquired on 35 pediatric patients using a 3T system in 35 pediatric patients. The images were graded alongside each other by using a 7-point Likert scale as follows: 1, nondiagnostic; 2, poor; 3, acceptable; 4, standard; 5, above average; 6, good; and 7, outstanding.The following were the average scores for EPI and RS-EPI, respectively: resolution, 3.5/5.2; distortion level, 2.9/6.0; SNR, 3.4/4.1; lesion conspicuity, 3.3/5.9; and diagnostic confidence, 3.2/6.0. Overall, the RS-EPI had significantly improved diagnostic confidence and more reliably defined the extent and structure of several lesions. Although ASSET EPI scans had better SNR per scanning time, the higher spatial resolution as well as reduced blurring and distortions on RS-EPI scans helped to better reveal important anatomic details at the cortical-subcortical levels, brain stem, temporal and inferior frontal lobes, skull base, sinonasal cavity, cranial nerves, and orbits.This work shows the importance of both resolution and decreased distortions in the clinics, which can be accomplished by a combination of parallel imaging and alternative k-space trajectories such as RS-EPI.
View details for DOI 10.3174/ajnr.A2481
View details for Web of Science ID 000294275100023
View details for PubMedID 21596809
Liposomal cytarabine for central nervous system embryonal tumors in children and young adults JOURNAL OF NEURO-ONCOLOGY 2011; 103 (3): 561-566
To assess the tolerability and efficacy of liposomal cytarabine (LC), an encapsulated, sustained-release, intrathecal (IT) formulation of cytosine arabinoside, in de novo and relapsed central nervous system (CNS) embryonal tumors in children and young adults. We studied retrospectively all patients less than age 30 at our institution treated consecutively with LC for medulloblastoma (MB), primitive neuroectodermal tumor (PNET), and atypical teratoid rhabdoid tumor (ATRT). Seventeen patients received LC (2 mg/kg up to 50 mg, every 2 weeks to monthly) at diagnosis of high-risk CNS embryonal tumor (2 PNET, 3 ATRT) or relapse of MB (12 MB; 9 had leptomeningeal metastases). Sixteen patients received concurrent systemic chemotherapy. A total of 108 doses were administered (IT 82, intraventricular 26) with a mean of six (range 1-16) treatments per patient. Only three administrations were associated with adverse effects of arachnoiditis or headache. None developed malignant cerebrospinal fluid (CSF) cytology while receiving LC. All the six evaluable patients with malignant CSF cytology and treated with at least two doses cleared their CSF (mean 3 doses, range 1-5). Median overall survival in relapse patients was 9.1 months. Five patients (4 de novo and 1 relapsed) remain alive in complete remission for a median 26.8 months from first LC. Liposomal cytarabine is an easily administered, well-tolerated, and active drug in patients with high-risk embryonal neoplasms. One-third of our cohort remains in remission from otherwise fatal diagnoses. Our findings warrant a phase II trial of LC in newly diagnosed or recurrent CNS embryonal tumors.
View details for DOI 10.1007/s11060-010-0419-y
View details for Web of Science ID 000291703000018
View details for PubMedID 20859651
Imaging of Nonaccidental Injury and the Mimics: Issues and Controversies in the Era of Evidence-Based Medicine RADIOLOGIC CLINICS OF NORTH AMERICA 2011; 49 (1): 205-?
Because of the controversy involving the determination of child abuse, or nonaccidental injury (NAI), radiologists must be familiar with the issues, literature, and principles of evidence-based medicine to understand the role of imaging. Children with suspected NAI must receive protective evaluation along with a timely and complete clinical and imaging work-up. Imaging findings cannot stand alone and must be correlated with clinical findings, laboratory testing, and pathologic and forensic examinations. Only the child protection investigation may provide the basis for inflicted injury in the context of supportive clinical, imaging, biomechanical, or pathology findings.
View details for DOI 10.1016/j.rcl.2010.08.001
View details for Web of Science ID 000285850900011
View details for PubMedID 21111136
Septopreoptic Holoprosencephaly: A Mild Subtype Associated with Midline Craniofacial Anomalies AMERICAN JOURNAL OF NEURORADIOLOGY 2010; 31 (9): 1596-1601
HPE is a congenital brain malformation characterized by failure of the prosencephalon to divide into 2 hemispheres. We have identified 7 patients who have a mild subtype of HPE in which the midline fusion was restricted to the septal region or preoptic region of the telencephalon. This subtype, which we call septopreoptic HPE, falls in the spectrum of lobar HPE, but lacks significant frontal neocortical fusion seen in lobar HPE. Other imaging characteristics include thickened or dysplastic fornix, absent or hypoplastic anterior CC, and single unpaired ACA. The SP was fully formed in 4, partially formed in 2, and absent in 1. Mild midline craniofacial malformation, such as SMMCI and CNPAS were found in 86% and 71%, respectively. Patients outside of infancy often manifested language delay, learning disabilities, or behavioral disturbances, while motor function was relatively spared.
View details for DOI 10.3174/ajnr.A2123
View details for Web of Science ID 000283011300009
View details for PubMedID 20488907
Infant Acute Life-Threatening Event-Dysphagic Choking Versus Nonaccidental Injury SEMINARS IN PEDIATRIC NEUROLOGY 2010; 17 (1): 7-11
A 4-month-old male infant presented to the emergency room with a history of choking while bottle feeding at home, and was found by emergency medical services (EMS) to be apneic and pulseless. He subsequently developed disseminated intravascular coagulopathy and died. Computed tomography (CT) and magnetic resonance imaging (MRI) showed subdural hemorrhages (SDHs), subarachnoid hemorrhage (SAH), and retinal hemorrhages (RHs), along with findings of hypoxic-ischemic encephalopathy (HIE). The caretaker account appeared to be inconsistent with the clinical and imaging features, and a diagnosis of nonaccidental injury with "shaken baby syndrome" was made. The autopsy revealed diffuse anoxic central nervous system (CNS) changes with marked edema, SAH, and SDH, but no evidence of "CNS trauma." Although NAI could not be ruled out, the autopsy findings provided further evidence that the child's injury could result from a dysphagic choking type of acute life threatening event (ALTE) as consistently described by the caretaker.
View details for DOI 10.1016/j.spen.2010.01.005
View details for Web of Science ID 000286144400004
View details for PubMedID 20434683
Neuroimaging Advances in Holoprosencephaly: Refining the Spectrum of the Midline Malformation AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS 2010; 154C (1): 120-132
Holoprosencephaly (HPE) is a complex congenital brain malformation characterized by failure of the forebrain to bifurcate into two hemispheres, a process normally completed by the fifth week of gestation. Modern high-resolution brain magnetic resonance imaging (MRI) has allowed detailed analysis of the cortical, white matter, and deep gray structural anomalies in HPE in living humans. This has led to better classification of types of HPE, identification of newer subtypes, and understanding of the pathogenesis. Currently, there are four generally accepted subtypes of HPE: alobar, semilobar, lobar, and middle interhemispheric variant. These subtypes are defined primarily by the degree and region of neocortical nonseparation. Rather than there being four discrete subtypes of HPE, we believe that there is a continuum of midline neocortical nonseparation resulting in a spectrum disorder. Many patients with HPE fall within the border zone between the neighboring subtypes. In addition, there are patients with very mild HPE, where the nonseparation is restricted to the preoptic (suprachiasmic) area. In addition to the neocortex, other midline structures such as the thalami, hypothalamic nuclei, and basal ganglia are often nonseparated in HPE. The cortical and subcortical involvements in HPE are thought to occur due to a disruption in the ventral patterning process during development. The severity of the abnormalities in these structures determines the severity of the neurodevelopmental outcome and associated sequelae.
View details for DOI 10.1002/ajmg.c.30238
View details for Web of Science ID 000274471700015
View details for PubMedID 20104607
Are Paterson's critics too biased to recognize rickets? ACTA PAEDIATRICA 2010; 99 (9): 1282-1283
Resolution of syringomyelia after release of tethered cord SURGICAL NEUROLOGY 2009; 72 (6): 657-661
Syringomyelia is an abnormal cystic dilatation of the spinal cord caused by excessive accumulation of CSF. Patients can develop various neurologic deficits secondary to untreated syringomyelia, some of which can be permanent despite surgical intervention.The authors present a patient with syringomyelia, aortic coarctation, and tethered cord syndrome. Serial radiographic imaging demonstrated initial significant reduction of the thoracic syrinx after coarctation repair and release of tethered cord. However, subsequent follow-up imaging revealed partial recurrence.This case provides evidence of a possible cause-effect relationship between syringomyelia and tethered cord. It demonstrates the indication of surveillance imaging of the entire spine to ensure that all potential etiologies of syringomyelia are identified and treated. Furthermore, it illustrates the complex dynamic nature of syrinx physiology and reinforces the importance of serial follow-up studies after surgical intervention.
View details for DOI 10.1016/j.surneu.2009.05.016
View details for Web of Science ID 000279233900026
View details for PubMedID 19604546
Performance of PROPELLER relative to standard FSE T2-weighted imaging in pediatric brain MRI PEDIATRIC RADIOLOGY 2009; 39 (10): 1038-1047
T2-weighted fast spin-echo imaging (T2-W FSE) is frequently degraded by motion in pediatric patients. MR imaging with periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) employs alternate sampling of k-space to achieve motion reduction.To compare T2-W PROPELLER FSE (T2-W PROP) with conventional T2-W FSE for: (1) image quality; (2) presence of artefacts; and (3) ability to detect lesions.Ninety-five pediatric patients undergoing brain MRI (1.5 T) were evaluated with T2-W FSE and T2-W PROP. Three independent radiologists rated T2-W FSE and T2-W PROP, assessing image quality, presence of artefacts, and diagnostic confidence. Chi-square analysis and Wilcoxon signed rank test were used to assess the radiologists' responses.Compared with T2-W FSE, T2-W PROP demonstrated better image quality and reduced motion artefacts, with the greatest benefit in children younger than 6 months. Although detection rates were comparable for the two sequences, blood products were more conspicuous on T2-W FSE. Diagnostic confidence was higher using T2-W PROP in children younger than 6 months. Average inter-rater agreement was 87%.T2-W PROP showed reduced motion artefacts and improved diagnostic confidence in children younger than 6 months. Thus, use of T2-W PROP rather than T2-W FSE should be considered in routine imaging of this age group, with caution required in identifying blood products.
View details for DOI 10.1007/s00247-009-1292-8
View details for Web of Science ID 000269861000003
View details for PubMedID 19669747
Neonatal brain structure on MRI and diffusion tensor imaging, sex, and neurodevelopment in very-low-birthweight preterm children DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY 2009; 51 (7): 526-535
The neurological basis of an increased incidence of cerebral palsy (CP) in preterm males is unknown. This study examined neonatal brain structure on magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) at term-equivalent age, sex, and neurodevelopment at 1 year 6 months on the basis of the Amiel-Tison neurological examination, Gross Motor Function Classification System, and Bayley Scales of Infant Development in 78 very-low-birthweight preterm children (41 males, 37 females; mean gestational age 27.6 wks, SD 2.5; mean birthweight 1021 g, SD 339). Brain abnormalities on MRI and DTI were not different between males and females except in the splenium of the corpus callosum, where males had lower DTI fractional anisotropy (p=0.025) and a higher apparent diffusion coefficient (p=0.013), indicating delayed splenium development. In the 26 infants who were at higher risk on the basis of DTI, males had more abnormalities on MRI (p=0.034) and had lower fractional anisotropy and a higher apparent diffusion coefficient in the splenium (p=0.049; p=0.025) and right posterior limb of the internal capsule (PLIC; p=0.003; p=0.033). Abnormal neurodevelopment was more common in males (n=9) than in females (n=2; p=0.036). Children with abnormal neurodevelopment had more abnormalities on MRI (p=0.014) and reduced splenium and right PLIC fractional anisotropy (p=0.001; p=0.035). In children with abnormal neurodevelopment, right PLIC fractional anisotropy was lower than left (p=0.035), whereas in those with normal neurodevelopment right PLIC fractional anisotropy was higher than left (p=0.001). Right PLIC fractional anisotropy correlated to neurodevelopment (rho=0.371, p=0.002). Logistic regression predicted neurodevelopment with 94% accuracy; only right PLIC fractional anisotropy was a significant logistic coefficient. Results indicate that the higher incidence of abnormal neurodevelopment in preterm males relates to greater incidence and severity of brain abnormalities, including reduced PLIC and splenium development.
View details for DOI 10.1111/j.1469-8749.2008.03231.x
View details for Web of Science ID 000266696900007
View details for PubMedID 19459915
Salivary Gland Anlage Tumor in a Neonate Presenting with Respiratory Distress: Radiographic and Pathologic Correlation AMERICAN JOURNAL OF NEURORADIOLOGY 2009; 30 (5): 1022-1023
We present a case of congenital salivary gland anlage tumor (SGAT) of the nasal septum in a 2-week-old infant who had difficulty breathing through her nose since birth. CT and MR imaging demonstrated a circumscribed mass within the nasal cavity that did not communicate with the intracranial compartment. Differential diagnosis and clinical significance of recognizing this rare lesion are reviewed.
View details for DOI 10.3174/ajnr.A1364
View details for Web of Science ID 000266133000027
View details for PubMedID 19112069
Traumatic Spinal Cord Injury: Accidental Versus Nonaccidental Injury SEMINARS IN PEDIATRIC NEUROLOGY 2008; 15 (4): 178-184
A 21-month-old boy with steroid-dependent asthma presented to the emergency room with Glascow Coma Score (GCS) 3 and retinal hemorrhages. He was found to have subdural and subarachnoid hemorrhage on computed tomography plus findings of hypoxic-ischemic encephalopathy (HIE). The caretaker history was thought to be inconsistent with the clinical and imaging features, and the patient was diagnosed with nonaccidental injury (NAI) and "shaken baby syndrome." The autopsy revealed a cranial impact site and fatal injury to the cervicomedullary junction. Biomechanical analysis provided further objective support that, although NAI could not be ruled out, the injuries could result from an accidental fall as consistently described by the caretaker.
View details for DOI 10.1016/j.spen.2008.10.009
View details for Web of Science ID 000207789900011
View details for PubMedID 19073323
Rickets vs. abuse: a national and international epidemic PEDIATRIC RADIOLOGY 2008; 38 (11): 1210-1216
Correlation of Clinical and Biochemical Findings with Diabetic Ketoacidosis-Related Cerebral Edema in Children Using Magnetic Resonance Diffusion-Weighted Imaging JOURNAL OF PEDIATRICS 2008; 153 (4): 541-546
To determine clinical and biochemical factors influencing cerebral edema formation during diabetic ketoacidosis (DKA) in children.We used magnetic resonance diffusion-weighted imaging to quantify edema formation. We measured the apparent diffusion coefficient (ADC) of brain water during and after DKA treatment in 26 children and correlated ADC changes with clinical and biochemical variables.Mean ADC values were elevated during DKA treatment compared with baseline (8.13 +/- 0.47 vs 7.74 +/- 0.49 x 10(-4) mm(2)/sec, difference in means 0.40, 95% CI: 0.25 to 0.55, P < .001). Children with altered mental status during DKA had greater elevation in ADC. ADC elevation during DKA was positively correlated with initial serum urea nitrogen concentration (correlation coefficient 0.41, P = .03) and initial respiratory rate (correlation coefficient 0.61, P < .001). ADC elevation was not significantly correlated with initial serum glucose, sodium or effective osmolality, nor with changes in glucose, sodium or osmolality during treatment. Multivariable analyses identified the initial urea nitrogen concentration and respiratory rate as independently associated with ADC elevation.The degree of edema formation during DKA in children is correlated with the degree of dehydration and hyperventilation at presentation, but not with factors related to initial osmolality or osmotic changes during treatment. These data support the hypothesis that CE is related to cerebral hypoperfusion during DKA, and that osmotic fluctuations during DKA treatment do not play a primary causal role.
View details for DOI 10.1016/j.jpeds.2008.04.018
View details for Web of Science ID 000260101600023
View details for PubMedID 18589447
Can magnetic resonance spectroscopy predict neurodevelopmental outcome in very low birth weight preterm infants? JOURNAL OF PERINATOLOGY 2008; 28 (9): 611-618
Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children JOURNAL OF PEDIATRIC SURGERY 2008; 43 (3): 451-460
The authors report their experience with doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations (LMs) in children.A retrospective chart review was used to collect data on 11 patients treated with doxycycline sclerotherapy for LMs of the head and neck at our institution since 2003. Radiographic imaging allowed classification of patient LM as macrocystic, microcystic, or mixed according to previously published guidelines. Only patients with macrocystic or mixed lesions were offered doxycycline sclerotherapy. Radiographic imaging and physical examination were used to determine efficacy of treatment. After each treatment, the clinical and radiographic response was characterized as excellent (> or = 95% decrease in lesion size), satisfactory (> or = 50% decrease in volume and asymptomatic), or poor (< 50% decrease in volume or symptomatic).Eleven patients underwent a total of 23 sclerotherapies with an average of 2 treatments per patient (range, 1-4). All 7 patients with macrocystic lesions achieved complete clinical resolution with an average radiographic resolution of 93%. The 4 patients with mixed lesions achieved only partial clinical resolution and an average of 73% radiographic resolution. No patient experienced any adverse effects related to the treatment. At a median follow-up of 8 months, 2 patients (18%) experienced lesion recurrence in the setting of concomitant infection.Doxycycline sclerotherapy is safe and effective as a primary treatment modality for macrocystic and mixed LMs of the head and neck in the pediatric population.
View details for DOI 10.1016/j.jpedsurg.2007.10.009
View details for Web of Science ID 000254803500008
View details for PubMedID 18358281
Utility of temporal bone computed tomographic measurements in the evaluation of inner ear malformations ARCHIVES OF OTOLARYNGOLOGY-HEAD & NECK SURGERY 2008; 134 (1): 50-56
To investigate whether normative inner ear measurements can assist in the evaluation of sensorineural hearing loss (SNHL).Retrospective cohort review.A tertiary care hospital.Computed tomography or magnetic resonance imaging was performed on 188 ears with SNHL and 220 ears without SNHL (204 children) between 2001 and 2004.Two readers measured the basal turn of cochlea (BTC) lumen, lateral semicircular canal (LSCC) bony island width, superior semicircular canal (SSCC) bony island width, and cochlear height (CH).A t test was performed comparing measurements in patients with and without SNHL. Interobserver variability was characterized by intraclass correlation coefficients and Bland-Altman plots.The t test results demonstrated no statistically significant differences between inner ear measurements in those with and without SNHL. The intraclass correlation coefficients for BTC lumen, CH, LSCC bony island width, and SSCC bony island width measurements was 0.612, 0.632, 0.869, and 0.912, respectively. Bland-Altman plots revealed systematic biases of 1%, 8%, 10%, and 21% for the BTC lumen, SSCC bony island width, LSCC bony island width, and CH measurements, respectively.Inner ear measurements in children with and without SNHL are not statistically different. Moreover, the measurements are difficult to interpret because while they demonstrate good reproducibility, they are susceptible to systematic biases. However, use of inner ear measurements is more sensitive in identifying vestibulocochlear dysplasias and should be considered to complement visual analysis.
View details for Web of Science ID 000252313300010
View details for PubMedID 18209137
Congenital glioblastoma multiforme: Case report and review of the literature PEDIATRIC NEUROSURGERY 2008; 44 (4): 304-312
Congenital glioblastoma multiforme is a rare primary brain tumor that has a unique biology distinct from pediatric and adult variants. In this report, we present a case of congenital glioblastoma with complicated management course. A literature review of previously reported cases is included to illustrate the epidemiology and natural history of this disease. A 9-month-old male infant developed acute lethargy, hemiparesis and unilaterally dilated pupil. Imaging studies revealed a large hemispheric tumor, resulting in significant midline shift suggestive of impending herniation. Emergent tumor cystic fluid drainage was performed at initial presentation. A frontotemporoparietal craniotomy was performed on the following day to attempt a gross total resection. Adjuvant chemotherapy consisting of oral temozolomide was administered. The patient eventually succumbed 4 months later due to aggressive tumor progression. Congenital glioblastoma should be included in the differential diagnosis of infants with large intracranial tumors. Although surgical intervention may increase survival, the overall outcome remains poor despite maximal multimodal treatment.
View details for DOI 10.1159/000134922
View details for Web of Science ID 000258318800008
View details for PubMedID 18504417
Neonatal microstructural development of the internal capsule on diffusion tensor imaging correlates with severity of gait and motor deficits DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY 2007; 49 (10): 745-750
Neonatal microstructural development in the posterior limbs of the internal capsule (PLIC) was assessed using diffusion tensor imaging (DTI) fractional anisotropy (FA) in 24 very-low-birthweight preterm infants at 37 weeks' gestational age and compared with the children's gait and motor deficits at 4 years of age. There were 14 participants with normal neonatal FA values (seven females, seven males; born at 27.6 weeks [SD 2.3] gestational age; birthweight 1027g [SD 229]) and 10 participants with low FA values in the PLIC (four females, six males; born at 28.4 weeks [SD 2.0] gestational age; birthweight 1041g [SD 322]). Seven of the 10 children with low FA and none of the children with normal FA had been diagnosed with CP by the time of gait testing. Among children with low neonatal FA, there was a strong negative correlation between FA of the combined left and right side PLIC and log NI (r=-0.89, p=0.001) and between FA and GMFCS (r=-0.65, p=0.04) at 4 years of age. There was no correlation between FA and gait NI or GMFCS at 4 years of age among children with normal neonatal FA. This preliminary study suggests neonatal DTI may be an important predictor of the severity of future gait and motor deficits.
View details for Web of Science ID 000249660400007
View details for PubMedID 17880643
Cerebral proton magnetic resonance spectroscopy in children with diabetic ketoacidosis AMERICAN JOURNAL OF NEURORADIOLOGY 2007; 28 (5): 895-899
Subclinical cerebral edema occurs in many, if not most, children with diabetic ketoacidosis (DKA) and may be an indicator of subtle brain injury. Brain ratios of N-acetylaspartate (NAA) to creatine (Cr), measured by proton MR spectroscopy, decrease with neuronal injury or dysfunction. We hypothesized that brain NAA/Cr ratios may be decreased in children in DKA, indicating subtle neuronal injury.Twenty-nine children with DKA underwent cerebral proton MR spectroscopy during DKA treatment (2-12 hours after initiating therapy) and after recovery from the episode (72 hours or more after the initiation of therapy). We measured peak heights of NAA, Cr, and choline (Cho) in 3 locations within the brain: the occipital gray matter, the basal ganglia, and periaqueductal gray matter. These regions were identified in previous studies as areas at greater risk for neurologic injury in DKA-related cerebral edema. We calculated the ratios of NAA/Cr and Cho/Cr and compared these ratios during the acute illness and recovery periods.In the basal ganglia, the ratio of NAA/Cr was significantly lower during DKA treatment compared with that after recovery (1.68 +/- 0.24 versus 1.86 +/- 0.28, P<.005). There was a trend toward lower NAA/Cr ratios during DKA treatment in the periaqueductal gray matter (1.66 +/- 0.38 versus 1.91 +/- 0.50, P=.06) and the occipital gray matter (1.97 +/- 0.28 versus 2.13 +/- 0.18, P=.08). In contrast, there were no significant changes in Cho/Cr ratios in any region.NAA/Cr ratios are decreased in children during DKA and improve after recovery. This finding suggests that during DKA neuronal function or viability or both are compromised and improve after treatment and recovery.
View details for Web of Science ID 000246601600028
View details for PubMedID 17494665
Imaging of the central nervous system in suspected or alleged nonaccidental injury, including the mimics. Topics in magnetic resonance imaging 2007; 18 (1): 53-74
Because of the widely acknowledged controversy in nonaccidental injury, the radiologist involved in such cases must be thoroughly familiar with the imaging, clinical, surgical, pathological, biomechanical, and forensic literature from all perspectives and with the principles of evidence-based medicine. Children with suspected nonaccidental injury versus accidental injury must not only receive protective evaluation but also require a timely and complete clinical and imaging workup to evaluate pattern of injury and timing issues and to consider the mimics of abuse. All imaging findings must be correlated with clinical findings (including current and past medical record) and with laboratory and pathological findings (eg, surgical, autopsy). The medical and imaging evidence, particularly when there is only central nervous system injury, cannot reliably diagnose intentional injury. Only the child protection investigation may provide the basis for inflicted injury in the context of supportive medical, imaging, biomechanical, or pathological findings.
View details for PubMedID 17607143
Macrocephaly, increased intracranial pressure, and hydrocephalus in the infant and young child. Topics in magnetic resonance imaging 2007; 18 (1): 31-51
Macrocephaly, increased intracranial pressure, and hydrocephalus are common related conditions that lead to cross-sectional imaging of the infant and young child. Imaging plays a central role in establishing the diagnosis and guiding disposition and treatment of these patients. In this review, a general overview is provided, and the more common causes of hydrocephalus are presented, including posthemorrhage, postinfection, developmental malformations, and masses. Imaging guidelines are also outlined for initial evaluation and follow-up, along with a discussion of the imaging features of shunt malfunction.
View details for PubMedID 17607142
A syndrome of irreversible leukoencephalopathy following pediatric allogeneic bone marrow transplantation PEDIATRIC BLOOD & CANCER 2007; 48 (2): 213-217
Despite decreases in overall mortality following bone marrow transplantation (BMT), a number of complications such as neurotoxicity have been described and often associated with immunosuppressive agents. The syndrome of reversible posterior leukoencephalopathy has been described in patients receiving cyclosporin and FK-506. We report here a subset of children who developed a syndrome of previously undescribed irreversible leukoencephalopathy following allogeneic BMT.Between 1996 and 2002, 138 pediatric patients received an allogeneic BMT at Lucile Salter Packard Children's Hospital at Stanford. Six cases of irreversible leukoencephalopathy were observed. Cases were defined as children who exhibited progressive and continued, severe neurologic deterioration lasting greater than 2 weeks and consistent with non-localizing, central nervous system abnormalities. Medical records and magnetic resonance images (MRIs) were reviewed.Median age of the affected patients at BMT was 7.8 years. All six received cyclosporine, and [corrected] one had elevated drug levels. Encephalopathy occurred at a median of 53 days (range 14-77) following BMT. Symptoms at onset of leukoenceophalopathy included confusion and altered mental status, sluggish pupillary responses, abnormal movements, and seizures. Two patients died during their neurologic decline. Four patients remain alive with persistent encephalopathy. MRI showed abnormalities in all patients including periventricular or subcortical white matter involvement in all, and basal ganglia lesions in three.We report a syndrome of irreversible neurologic deficits and cerebral white matter abnormalities following allogeneic BMT, yet not associated with elevated cyclosporin levels. A precise mechanism for this syndrome is lacking and warrants further consideration.
View details for DOI 10.1002/pbc.20731
View details for Web of Science ID 000242875800016
View details for PubMedID 16365853
Frequency of sub-clinical cerebral edema in children with diabetic ketoacidosis PEDIATRIC DIABETES 2006; 7 (2): 75-80
Symptomatic cerebral edema occurs in approximately 1% of children with diabetic ketoacidosis (DKA). However, asymptomatic or subclinical cerebral edema is thought to occur more frequently. Some small studies have found narrowing of the cerebral ventricles indicating cerebral edema in most or all children with DKA, but other studies have not detected narrowing in ventricle size. In this study, we measured the intercaudate width of the frontal horns of the lateral ventricles using magnetic resonance imaging (MRI) in children with DKA during treatment and after recovery from the DKA episode. We determined the frequency of ventricular narrowing and compared clinical and biochemical data for children with and without ventricular narrowing. Forty-one children completed the study protocol. The lateral ventricles were significantly smaller during DKA treatment (mean width, 9.3 +/- 0.3 vs. 10.2 +/- 0.3 mm after recovery from DKA, p < 0.001). Children with ventricular narrowing during DKA treatment (22 children, 54%) were more likely to have mental status abnormalities than those without narrowing [12/22 vs. 4/19 with Glasgow Coma Scale (GCS) scores below 15 during therapy, p = 0.03]. Multiple logistic regression analysis revealed that a lower initial PCO2 level was significantly associated with ventricular narrowing [odds ratio (OR) = 0.88, 95% confidence interval (95% CI) = 0.78-0.99, p = 0.047). No other variables analyzed were associated with ventricular narrowing in the multivariate analysis. We conclude that narrowing of the lateral ventricles is evident in just over half of children being treated for DKA. Although children with ventricular narrowing did not exhibit neurological abnormalities sufficient for a diagnosis of 'symptomatic cerebral edema', mild mental status abnormalities occurred frequently, suggesting that clinical evidence of cerebral edema in children with DKA may be more common than previously reported.
View details for Web of Science ID 000236962500002
View details for PubMedID 16629712
Radiotherapy in pediatric medulloblastoma: Quality assessment of Pediatric Oncology Group Trial 9031 INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 2006; 64 (5): 1325-1330
To evaluate the potential influence of radiotherapy quality on survival in high-risk pediatric medulloblastoma patients.Trial 9031 of the Pediatric Oncology Group (POG) aimed to study the relative benefit of cisplatin and etoposide randomization of high-risk patients with medulloblastoma to preradiotherapy vs. postradiotherapy treatment. Two-hundred and ten patients were treated according to protocol guidelines and were eligible for the present analysis. Treatment volume (whole brain, spine, posterior fossa, and primary tumor bed) and dose prescription deviations were assessed for each patient. An analysis of first site of failure was undertaken. Event-free and overall survival rates were calculated. A log-rank test was used to determine the significance of potential survival differences between patients with and without major deviations in the radiotherapy procedure.Of 160 patients who were fully evaluable for all treatment quality parameters, 91 (57%) had 1 or more major deviations in their treatment schedule. Major deviations by treatment site were brain (26%), spinal (7%), posterior fossa (40%), and primary tumor bed (17%). Major treatment volume or total dose deviations did not significantly influence overall and event-free survival.Despite major treatment deviations in more than half of fully evaluable patients, underdosage or treatment volume misses were not associated with a worse event-free or overall survival.
View details for DOI 10.1016/j.ijrobp.2005.11.002
View details for Web of Science ID 000236477200005
View details for PubMedID 16413699
Metabolite ratios measured by proton magnetic resonance spectroscopy correlate with postmenstrual age in very low birth weight preterm infants with normal neuromotor and Bayley results. LIPPINCOTT WILLIAMS & WILKINS. 2006: S97-S97
Magnetic resonance imaging of the kinked fetal brain stem - A sign of severe dysgenesis JOURNAL OF ULTRASOUND IN MEDICINE 2005; 24 (12): 1697-1709
Magnetic resonance imaging (MRI) allows visualization of the fetal brain stem in a manner not previously possible. A "kinked" brain stem is a sign of severe neurodysgenesis. The purpose of this series was to describe cases of a kinked brain stem detected on prenatal MRI and to discuss the possible genetic and syndromic etiologies.Seven cases of a kinked brain stem on fetal MRI (gestational age range, 18-34 weeks) were reviewed and correlated with other clinical, genetic, imaging, and autopsy findings.In all cases, there was associated cerebellar hypogenesis. Additional findings were ventriculomegaly (4 cases), cerebral hypogenesis (3 cases), microcephaly (4 cases), schizencephaly (1 case), cephalocele (1 case), hypogenesis of the corpus callosum (1 case), and hydrocephalus (1 case). In 2 cases, prenatal sonography misidentified the kinked brain stem as the cerebellum.A kinked brain stem is an indicator of severe neurodysgenesis arising early in gestation. Magnetic resonance imaging provides the necessary resolution to detect this sign and delineate any associated anomalies in utero to assist with further genetic evaluation, management, and counseling.
View details for Web of Science ID 000234064500011
View details for PubMedID 16301726
Quantitative analysis of the corpus callosum in children with cerebral palsy and developmental delay: correlation with cerebral white matter volume PEDIATRIC RADIOLOGY 2005; 35 (12): 1199-1207
The direct quantitative correlation between thickness of the corpus callosum and volume of cerebral white matter in children with cerebral palsy and developmental delay has not been demonstrated.This study was conducted to quantitatively correlate the thickness of the corpus callosum with the volume of cerebral white matter in children with cerebral palsy and developmental delay.A clinical database of 70 children with cerebral palsy and developmental delay was established with children between the ages of 1 and 5 years. These children also demonstrated abnormal periventricular T2 hyperintensities associated with and without ventriculomegaly. Mid-sagittal T1-weighted images were used to measure the thickness (genu, mid-body, and splenium) and length of the corpus callosum. Volumes of interest were digitized based on gray-scale densities to define the hemispheric cerebral white matter on axial T2-weighted and FLAIR images. The thickness of the mid-body of the corpus callosum was correlated with cerebral white matter volume. Subgroup analysis was also performed to examine the relationship of this correlation with both gestational age and neuromotor outcome. Statistical analysis was performed using analysis of variance and Pearson correlation coefficients.There was a positive correlation between the thickness of the mid-body of the corpus callosum and the volume of cerebral white matter across all children studied (R=0.665, P=0.0001). This correlation was not dependent on gestational age. The thickness of the mid-body of the corpus callosum was decreased in the spastic diplegia group compared to the two other groups (hypotonia and developmental delay only; P<0.0001). Within each neuromotor subgroup, there was a positive correlation between thickness of the mid-body of the corpus callosum and volume of the cerebral white matter.The thickness of the mid-body of the corpus callosum positively correlates with volume of cerebral white matter in children with cerebral palsy and developmental delay, regardless of gestational age or neuromotor outcome. Assessment of the thickness of the corpus callosum might help in estimating the extent of the loss of volume of cerebral white matter in children with a broad spectrum of periventricular white matter injury.
View details for DOI 10.1007/s00247-005-1577-5
View details for Web of Science ID 000233527800006
View details for PubMedID 16136325
Cortical magnetic resonance imaging findings in familial pediatric bipolar disorder BIOLOGICAL PSYCHIATRY 2005; 58 (3): 197-203
Morphometric magnetic resonance imaging (MRI) studies of pediatric bipolar disorder (BD) have not reported on gray matter volumes but have reported increased lateral ventricular size and presence of white matter hyperintensities (WMH). We studied gray matter volume, ventricular-to-brain ratios (VBR), and number of WMH in patients with familial, pediatric BD compared with control subjects.Twenty subjects with BD (aged 14.6 +/- 2.8 years; 4 female) according to the Washington University in St. Louis Kiddie Schedule for Affective Disorders and Schizophrenia, each with a parent with BD, and 20 age-, gender-, and intelligence quotient-matched healthy control subjects (aged 14.1 +/- 2.8 years; 4 female) were scanned at 3 T. Most subjects were taking psychotropic medications. A high-resolution T1-weighted spoiled gradient echo three-dimensional MRI sequence was analyzed by BrainImage for volumetric measurements, and T2-weighted images were read by a neuroradiologist to determine presence of WMH.After covarying for age and total brain volume, there were no significant differences between subjects with BD and control subjects in volume of cerebral (p = .09) or prefrontal gray matter (p = .34). Subjects with BD did not have elevated numbers of WMH or greater VBR when compared with control subjects.Children and adolescents with familial BD do not seem to have decreased cerebral grey matter or increased numbers of WMH, dissimilar to findings in adults with BD. Gray matter decreases and development of WMH might be later sequelae of BD or unique to adult-onset BD.
View details for DOI 10.1016/j.biopsych.2005.03.039
View details for Web of Science ID 000231057100003
View details for PubMedID 16084840
Detection of cerebral beta-hydroxy butyrate, acetoacetate, and lactate on proton MR spectroscopy in children with diabetic ketoacidosis AMERICAN JOURNAL OF NEURORADIOLOGY 2005; 26 (5): 1286-1291
Ketone bodies provide important alternate fuel for brain metabolism, and their transport into the brain increases with prolonged fasting. During diabetic ketoacidosis (DKA), serum ketone concentrations markedly increase; however, little is known about whether ketone bodies accumulate in cerebral tissues during DKA. We used proton MR spectroscopy (MRS) to detect cerebral beta-hydroxy butyrate (betaOHB) and acetone/acetocaetate (AcAc) in children with DKA.Twenty-five children underwent brain MRS: nine within 4 hours of the start of treatment for DKA; 11, at 4-8 hours; and five, at 8-12 hours. MRS was repeated after their recovery from the DKA episode at > or =72 hours after the start of treatment. MRS was evaluated for peaks corresponding to betaOHB (doublet centered on 1.20 ppm) and lactate (doublet centered on 1.33 ppm). Difference spectroscopy was used to identify the AcAc peak at 2.22-2.26 ppm.betaOHB was detected in 13 children (52%), more frequently within 4 hours (eight children, 89%) than after 4 hours (five children, 31%). AcAc was detected in 15 children (60%), more frequently at >4 hours after the start of treatment (12 patients, 75%) than in the first 4 hours (three patients, 33%). Lactate was detected in five children (18%), all within the first 8 hours of treatment.In children, betaOHB and AcAc accumulate in the brain during DKA, and they can be detected on MRS. Care should be taken in interpreting MRS results in patients with DKA to avoid erroneously attributing betaOHB peaks to lactate.
View details for Web of Science ID 000229306100053
View details for PubMedID 15891198
Lateral meningocele syndrome: Vertical transmission and expansion of the phenotype AMERICAN JOURNAL OF MEDICAL GENETICS PART A 2005; 133A (2): 115-121
Lateral meningoceles were first described by Lehman et al. [(1977); J Pediatr 90: 49-54] in a patient with other skeletal findings and distinctive craniofacial features. Subsequently, six more patients with the so-called lateral meningocele syndrome (LMS) have been reported. We describe the findings in three new cases and expand the phenotype. The existence of an affected mother and daughter supports the hypothesis that LMS is a dominant disorder affecting primarily the connective tissue.
View details for DOI 10.1002/ajmg.a.30526
View details for Web of Science ID 000227194100001
View details for PubMedID 15666314
Neonatal brain magnetic resonance imaging before discharge is better than serial cranial ultrasound in predicting cerebral palsy in very low birth weight preterm infants PEDIATRICS 2004; 114 (4): 992-998
To compare the value of serial cranial ultrasound (US) with a single magnetic resonance imaging (MRI) before discharge in very low birth weight preterm infants to predict cerebral palsy (CP).Infants who weighed <1250 g at birth and were <30 weeks' gestational age underwent conventional brain MRI at near term (36-40 weeks' postmenstrual age) using 1.5 Tesla MRI scanner. Sagittal and axial T1 and T2 fluid attenuated inversion recovery and gradient recalled echo images were obtained. Cranial US was also obtained at least twice during the first 2 weeks of life. MRI and US images were interpreted by 2 independent radiologists, who were masked to clinical outcome, and scored as follows: category 1, no abnormality; category 2, subependymal hemorrhage or mineralization; category 3, moderate to severe ventriculomegaly; category 4, focal parenchymal abnormality with or without ventriculomegaly. For the purpose of this study, 1 and 2 were categorized as "normal," and 3 and 4 were categorized as "abnormal." The infants were assessed at a mean age of 20 and 31 months using the Amiel-Tison standardized neurodevelopmental examination.The sensitivity and specificity of MRI for predicting CP were 71% and 91% at 20 month and 86% and 89% at 31 months, respectively. The sensitivity and specificity of US for predicting CP were 29% and 86% at 20 months and 43% and 82% at 31 months.As a predictor of outcome for CP, MRI at near-term in very low birth weight preterm neonates is superior to US. However, both US and MRI demonstrate high specificity.
View details for DOI 10.1542/peds.2003-0772-L
View details for Web of Science ID 000224242200034
View details for PubMedID 15466096
Mechanism of cerebral edema in children with diabetic ketoacidosis JOURNAL OF PEDIATRICS 2004; 145 (2): 164-171
Cerebral edema during diabetic ketoacidosis (DKA) has been attributed to osmotic cellular swelling during treatment. We evaluated cerebral water distribution and cerebral perfusion during DKA treatment in children.We imaged 14 children during DKA treatment and after recovery, using both diffusion and perfusion weighted magnetic resonance imaging (MRI). We assessed the apparent diffusion coefficients (ADCs) and measures reflecting cerebral perfusion.The ADC was significantly elevated during DKA treatment (indicating increased water diffusion) in all regions except the occipital gray matter. Mean reductions in the ADC from initial to postrecovery MRI were: basal ganglia 4.7 +/- 2.5 x 10(-5) mm(2)/s (P=.002), thalamus 3.7 +/- 2.8 x 10(-5) mm(2)/s, (P=.002), periaqueductal gray matter 4.3 +/- 5.1 x 10(-5) mm(2)/s (P=.03), and frontal white matter 2.0 +/- 3.1 x 10(-5) mm(2)/s (P=.03). In contrast, the ADC in the occipital gray matter increased significantly from the initial to postrecovery MRI (mean increase 3.9 +/- 3.9 x 10(-5) mm(2)/s, P=.004). Perfusion MRI during DKA treatment revealed significantly shorter mean transit times (MTTs) and higher peak tracer concentrations, possibly indicating increased cerebral blood flow (CBF).Elevated ADC values during DKA treatment suggests a vasogenic process as the predominant mechanism of edema formation rather than osmotic cellular swelling.
View details for DOI 10.1016/j.jpeds.2004.03.045
View details for Web of Science ID 000223406200008
View details for PubMedID 15289761
A sojourn in the abyss: Hypothesis, theory, and established truth in infant head injury PEDIATRICS 2004; 114 (1): 326-326
Spontaneous kicking patterns at 3 months corrected age in preterm infants with normal and abnormal neonatal brain MRI NATURE PUBLISHING GROUP. 2004: 413A-413A
Fetal stroke PEDIATRIC NEUROLOGY 2004; 30 (3): 151-162
Fetal stroke, or that which occurs between 14 weeks of gestation and the onset of labor resulting in delivery, has been associated with postnatal epilepsy, mental retardation, and cerebral palsy. The entity is caused by antenatal ischemic, thrombotic, or hemorrhagic injury. We present seven new cases of fetal stroke diagnosed in utero and review the 47 cases reported in the literature. Although risk factors could not be assigned to 50% of the fetuses with stroke, the most common maternal conditions associated with fetal stroke were alloimmune thrombocytopenia and trauma. Magnetic resonance imaging was optimal for identifying fetal stroke, and prenatal imaging revealed hemorrhagic lesions in over 90% of studies; porencephalies were identified in just 13%. Seventy-eight percent of cases with reported outcome resulted in either death or adverse neurodevelopmental outcome at ages 3 months to 6 years. Fetal stroke appears to have different risk factors, clinical characteristics, and outcomes than other perinatal or childhood stroke syndromes. A better understanding of those risk factors predisposing a fetus to cerebral infarction may provide a basis for future therapeutic intervention trials. Ozduman K, Pober BR, Barnes P, Copel JA, Ogle EA, Duncan CC, Ment LR. Fetal stroke.
View details for DOI 10.1016/j.pediatrneurol.2003.08.004
View details for Web of Science ID 000220512700001
View details for PubMedID 15033196
Fast MR imaging of fetal central nervous system abnormalities RADIOLOGY 2003; 229 (1): 51-61
To evaluate with respect to gestational age (GA) the effect of magnetic resonance (MR) imaging on changes in diagnosis, patient counseling, and case management regarding fetuses suspected of having central nervous system (CNS) anomalies.The authors compared images from 242 ultrasonographic (US) studies and 242 MR imaging studies of the CNS in 214 fetuses. Reference standards of postnatal physical examination, imaging, surgical, and autopsy findings were available in 171 (79.9%) fetuses. Referring physicians were surveyed on how MR imaging findings changed patient counseling or case management. Outcomes were compared with respect to GA. Statistical tests used were the Fisher exact test, Student t test, and analysis of variance.Confirmatory US findings were normal in 69 fetuses. MR imaging findings changed diagnosis in 46 of 145 (31.7%) fetuses with abnormal US findings. The mean GA of 72 of 145 fetuses with changes in maternal counseling (25.9 weeks) was significantly greater than that in 73 of 145 fetuses without changes in maternal counseling (22.6 weeks, P <.01). The mean GA of the 46 fetuses with changes in diagnosis (26.3 weeks) was significantly greater than that of the 99 fetuses with no major change in diagnosis (23.3 weeks, P <.01). There were 27 of 145 changes in case management, with no significant difference in mean GA of fetuses with and those without changes in case management. In fetuses with abnormal US findings, MR images were used to decide to terminate the pregnancy (n = 13; mean GA, 20.1 weeks), continue the pregnancy (n = 4; mean GA, 19.2 weeks), direct the mode and/or location of delivery (n = 5; mean GA, 30.5 weeks), and direct perinatal care (n = 5; mean GA, 30.2 weeks).When a CNS anomaly is detected or suspected at US, MR imaging may demonstrate additional findings that can alter diagnosis and case management. Changes in management are GA dependent.
View details for DOI 10.1148/radiol.2291020770
View details for Web of Science ID 000185424900012
View details for PubMedID 12920177
Diffusion tensor brain imaging findings at term-equivalent age may predict neurologic abnormalities in low birth weight preterm infants AMERICAN JOURNAL OF NEURORADIOLOGY 2003; 24 (8): 1646-1653
Low birth weight preterm infants are at high risk of brain injury, particularly injury to the white matter. Diffusion tensor imaging is thought to be more sensitive than conventional MR imaging for detecting subtle white matter abnormalities. The objective of this study was to examine whether diffusion tensor imaging could detect abnormalities that may be associated with later neurologic abnormalities in infants with otherwise normal or minimally abnormal conventional MR imaging findings.We prospectively studied 137 low birth weight (<1800 g) preterm infants. Neonatal conventional MR imaging and diffusion tensor imaging were performed near term-equivalent age before discharge, and neurologic development of the infants was later followed up at 18 to 24 months of age.Among the preterm infants who were fully studied, 63 underwent normal conventional MR imaging. Three of these infants developed cerebral palsy, and 10 others showed abnormal neurologic outcome. Diffusion tensor imaging results for these infants showed a significant reduction of fractional anisotropy in the posterior limb of the internal capsule in neurologically abnormal infants (including those with cerebral palsy) compared with control preterm infants with normal neurologic outcomes.These results suggest that neonatal diffusion tensor imaging may allow earlier detection of specific anatomic findings of microstructural abnormalities in infants at risk for neurologic abnormalities and disability. The combination of conventional MR imaging and diffusion tensor imaging may increase the predictive value of neonatal MR imaging for later neurologic outcome abnormalities and may become the basis for future interventional clinical studies to improve outcomes.
View details for Web of Science ID 000185400100031
View details for PubMedID 13679287
Cortical ependymoma - A case report and review PEDIATRIC NEUROSURGERY 2003; 39 (1): 50-54
The authors report a rare case of a cortical ependymoma in a 10-year-old boy. The patient presented with complex partial seizures and a well-circumscribed, right frontal cortical mass. Routine microscopy showed a glial tumor with diverse histologic features. Immunohistochemistry and electron microscopy were required to establish the definitive diagnosis of cortical ependymoma. Cortical-based pediatric brain tumors range from World Health Organization grade I to III lesions and require significantly different treatment and follow-up. This case illustrates the importance of establishing an accurate neuropathologic tissue diagnosis of all pediatric cortical tumors.
View details for DOI 10.1159/000070881
View details for Web of Science ID 000183529400012
View details for PubMedID 12784079
Hydranencephaly owing to twin-twin transfusion: Serial fetal ultrasonography and magnetic resonance imaging findings JOURNAL OF CHILD NEUROLOGY 2003; 18 (5): 367-370
We report a newborn girl with hydranencephaly. In the setting of a monochorionic twin pregnancy, one twin's demise was detected by ultrasonography at 18 weeks of gestation, apparently the result of a twin-twin transfusion. In the surviving twin, the evolution of ventriculomegaly, first noted at 18 weeks, to hydranencephaly at 27 weeks is documented by serial sonograms. These findings were confirmed with fetal and postnatal magnetic resonance imaging.
View details for Web of Science ID 000183405600013
View details for PubMedID 12822826
A phase I trial of etanidazole and hyperfractionated radiotherapy in children with diffuse brainstem glioma INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 2003; 55 (5): 1182-1185
To determine the toxicity and maximum tolerated dose of etanidazole administered concurrently with hyperfractionated radiation therapy (HRT) for children with brainstem glioma.Eighteen patients with brainstem glioma were treated with etanidazole and HRT on a dose escalation protocol (Phase I trial) between 1990 and 1996. All patients had MRI confirmation of diffuse pontine glioma and signs/symptoms of cranial nerve deficit, ataxia, or long tract signs of <6 months' duration. Cervicomedullary tumors were excluded. Patients (median age: 8.5 years; 11 males, 7 females) received HRT to the tumor volume plus a 2-cm margin with parallel-opposed 6-15-MV photons. The total dose was 66 Gy in 44 fractions (1.5 Gy b.i.d., with at least 6 h between fractions) for the first 3 patients and 63 Gy in 42 fractions for the subsequent 15 patients. Etanidazole was administered as a rapid i.v. infusion 30 min before the morning fraction of HRT. Planned doses of etanidazole were 1.8 g/m(2) x 17 doses (30.6 g/m(2)) at Step 1 to a maximum of 2.4 g/m(2) x 21 doses (50.4 g/m(2)) at Step 8. Dose escalation was planned with 3 patients at each of the 8 levels.Three patients were treated at each dose level except Level 2, on which only 1 patient was treated. The highest dose level achieved was Level 7, which delivered a total etanidazole dose of 46.2 g/m(2). Two patients were treated at this level, and both patients experienced Grade 3 toxicity in the form of a diffuse cutaneous rash. Three patients received a lower dose of 42 g/m(2) (dose Level 6) without significant toxicity, and this represents the maximum tolerated dose (MTD). There were 23 cases of Grade 1 toxicity (10 vomiting, 5 peripheral neuropathy, 2 rash, 2 constipation, 1 weight loss, 3 others), 11 cases of Grade 2 toxicity (4 vomiting, 2 skin erythema, 2 constipation, 1 arthralgia, 1 urinary retention, 1 hematologic), and 4 Grade 3 toxicities (2 rash, 1 vomiting, 1 skin desquamation). Grade 2 or 3 peripheral neuropathy was not seen at any dose level. The median survival from the start of treatment was 8.5 months (range: 3-58 months).The MTD of etanidazole in children receiving HRT for brainstem glioma is 42 g/m(2), with cutaneous rash as the dose-limiting toxicity. This is in contrast to the adult experience, which demonstrates a 24% lower MTD of 34 g/m(2) limited by peripheral neuropathy.
View details for DOI 10.1016/S0360-3016(02)04391-2
View details for Web of Science ID 000181803500007
View details for PubMedID 12654425
Spontaneous resolution of a prepontine arachnoid cyst - Case report and review of the literature PEDIATRIC NEUROSURGERY 2002; 37 (3): 152-157
Prepontine arachnoid cysts are rare developmental anomalies that occur almost exclusively in children. The symptomatic child typically suffers from hydrocephalus, visual impairment, endocrine dysfunction and/or cranial neuropathies. Some cysts, however, are discovered incidentally upon prenatal or postnatal imaging for other indications. While there is little doubt that surgical treatment should be initiated to help the symptomatic child, appropriate therapy for the asymptomatic patient is unclear. Although arachnoid cysts are often managed conservatively using serial imaging, the consequences of injury to surrounding structures with prepontine cysts often lowers the threshold for intervention. The natural history of asymptomatic prepontine arachnoid cysts is unknown. It has been reported that some cysts enlarge and cause symptoms, whereas others are stable for years. This report describes an index case of spontaneous resolution of a prepontine arachnoid cyst in a female infant over a 5-year period.
View details for Web of Science ID 000177787800006
View details for PubMedID 12187060
Practice parameter: neuroimaging of the neonate - Report of the quality standards subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society PEDIATRIC RADIOLOGY 2002; 32 (9): 620-620
Practice parameter: Neuroimaging of the neonate - Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society NEUROLOGY 2002; 58 (12): 1726-1738
The authors reviewed available evidence on neonatal neuroimaging strategies for evaluating both very low birth weight preterm infants and encephalopathic term neonates.Routine screening cranial ultrasonography (US) should be performed on all infants of <30 weeks' gestation once between 7 and 14 days of age and should be optimally repeated between 36 and 40 weeks' postmenstrual age. This strategy detects lesions such as intraventricular hemorrhage, which influences clinical care, and those such as periventricular leukomalacia and low-pressure ventriculomegaly, which provide information about long-term neurodevelopmental outcome. There is insufficient evidence for routine MRI of all very low birth weight preterm infants with abnormal results of cranial US.Noncontrast CT should be performed to detect hemorrhagic lesions in the encephalopathic term infant with a history of birth trauma, low hematocrit, or coagulopathy. If CT findings are inconclusive, MRI should be performed between days 2 and 8 to assess the location and extent of injury. The pattern of injury identified with conventional MRI may provide diagnostic and prognostic information for term infants with evidence of encephalopathy. In particular, basal ganglia and thalamic lesions detected by conventional MRI are associated with poor neurodevelopmental outcome. Diffusion-weighted imaging may allow earlier detection of these cerebral injuries.US plays an established role in the management of preterm neonates of <30 weeks' gestation. US also provides valuable prognostic information when the infant reaches 40 weeks' postmenstrual age. For encephalopathic term infants, early CT should be used to exclude hemorrhage; MRI should be performed later in the first postnatal week to establish the pattern of injury and predict neurologic outcome.
View details for Web of Science ID 000176429700004
View details for PubMedID 12084869
MR imaging appearance of fetal cerebral ventricular morphology RADIOLOGY 2002; 223 (3): 652-660
To elucidate further the magnetic resonance (MR) imaging appearance of fetal cerebral ventricles by comparing ultrasonographic (US) and MR images.A retrospective review of MR and US images was performed for 110 normal fetuses and 94 fetuses with central nervous system abnormalities to assess lateral ventricular morphology as having (a) a normal appearance, (b) mild, disproportionate dilatation of the occipital horns with overall preservation of ventricular morphology, (c) colpocephaly with or without normal orientation of the frontal horns, (d) abnormal orientation of the frontal horns without colpocephaly, (e) an angular appearance, (f) fused frontal horns, (g) global dilation, or (h) a distorted appearance. Ventricular morphology on US and MR images was compared and correlated with reference standard diagnoses.US and MR imaging classifications were concordant in 145 of 188 (77%) examinations. Mild disproportion of occipital horns with respect to frontal horns was seen only on MR images. This ventricular configuration was present in eight of 110 normal fetuses and in 10 of 16 fetuses with isolated mild ventriculomegaly (P <.001). An angular configuration of the lateral ventricles, which is seen in fetuses with neural tube defects (NTDs), was present on review of MR images in 11 fetuses and on US images in one fetus. The ventricles of fetuses with NTDs and angular ventricles (3-12 mm) were significantly smaller than those of fetuses with NTDs and global dilatation of the ventricles (13-25 mm; P <.05).Ventricular contours differ with differing diagnoses of central nervous system abnormalities.
View details for DOI 10.1148/radiol.2233011336
View details for Web of Science ID 000175757300010
View details for PubMedID 12034931
Ethical issues in imaging nonaccidental injury: child abuse. Topics in magnetic resonance imaging 2002; 13 (2): 85-93
One of the most controversial areas of nonaccidental injury is the medical diagnosis of inflicted central nervous system injury and its impact on medical, social, and legal outcomes for children and families. This review addresses the role of the neuroradiologist in the clinical care of the pediatric patient and as an expert medical witness in the area of nonaccidental injury.
View details for PubMedID 12055453
Intracranial hemorrhage in infants and children with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) PEDIATRICS 2002; 109 (1)
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia. Most cases are caused by mutations in the endoglin gene on chromosome 9 (HHT type 1) or the activin receptor-like kinase 1 gene on chromosome 12 (HHT type 2), which leads to telangiectases and arteriovenous malformations (AVM) of the skin, mucosa, and viscera. Epistaxis is the most frequent presentation. Visceral involvement includes pulmonary, gastrointestinal, and cerebral AVMs, which have been reported predominantly in adults. The purpose of this article is to describe 9 children who presented with intracranial hemorrhage (ICH) secondary to cerebral AVM. None of these children was suspected of having HHT before the incident, despite family histories of the disease.We report the first case of an ICH secondary to a cerebral AVM in a neonate confirmed to have HHT type 1 by molecular analysis. We also describe a series of 8 additional cases of ICH secondary to cerebral AVM in children presumed to have HHT. Examination of multiple affected members from each of these families, using well-accepted published criteria, confirmed the diagnosis of HHT. In addition, genetic linkage studies and/or mutation analysis identified endoglin as the disease-causing gene in 6 of these families. Autopsy, imaging studies, and/or surgery confirmed the presence of cerebral AVMs and ICH in all 9 cases.Our report shows that infants and children with a family history of HHT are at risk for sudden and catastrophic ICH. A preemptive diagnosis may potentially identify and prevent more serious sequelae.
View details for Web of Science ID 000173006600012
View details for PubMedID 11773580
Intracranial hemorrhage in children with hereditary hemorrhagic telangiectasia. CELL PRESS. 2001: 221-221
Volumetric brain differences in children with periventricular T2-signal hyperintensities: A grouping by gestational age at birth. AMERICAN JOURNAL OF ROENTGENOLOGY 2001; 177 (3): 695-702
The purpose of this study was to compare both the volumes of the lateral ventricles and the cerebral white matter with gestational age at birth of children with periventricular white matter (PVWM) T2-signal hyperintensities on MR images. The spectrum of neuromotor abnormalities associated with these hyperintensities was also determined.We retrospectively reviewed the MR images of 70 patients who were between the ages of 1 and 5 years and whose images showed PVWM T2-signal hyperintensities. The patients were divided into premature (n = 35 children) and term (n = 35) groups depending on their gestational age at birth. Volumetric analysis was performed on four standardized axial sections using T2-weighted images. Volumes of interest were digitized on the basis of gray-scale densities of signal intensities to define the hemispheric cerebral white matter and lateral ventricles. Age-adjusted comparisons of volumetric measurements between the premature and term groups were performed using analysis of covariance.The volume of the cerebral white matter was smaller in the premature group (54 +/- 2 cm(3)) than in the term group (79 +/- 3 cm(3), p < 0.0001). The volume of the lateral ventricles was greater among the patients in the premature group (30 +/- 2 cm(3)) than among those in the term group (13 +/- 1 cm(3), p < 0.0001). Fifty percent of all the premature children had spastic diplegia or quadriplegia. Thirty-two percent of all the term children had hypotonia. There were patients in both groups whose PVWM T2-signal hyperintensities did not correlate with any neuromotor abnormalities but were associated with seizures or developmental delays.The differences in volumetric measurements of cerebral white matter and lateral ventricles in children with PVWM T2-signal hyperintensities are related to their gestational age at birth. Several neurologic motor abnormalities are found in children with such hyperintensities.
View details for Web of Science ID 000170672000038
View details for PubMedID 11517078
Line scan diffusion tensor MRI of the cervical spinal cord in preterm infants JOURNAL OF MAGNETIC RESONANCE IMAGING 2001; 13 (6): 949-953
Line scan diffusion tensor magenetic resonance imaging (DT-MRI) of the cervical spinal cord was demonstrated in vivo for unsedated preterm (gestational age 24-30 weeks at birth), very low birthweight (birthweight 620-1300 g) infants at postmenstrual ages from 29-40 weeks. Scalar invariant measures of diffusion [apparent diffusion coefficient (ADC) and relative anisotropy (RA)] determined from a cervical cord region of interest in each case are reported, characterizing the maturational status of the normal third trimester and newborn spinal cord. Mean ADC of 11 infants was 1.2 +/- 0.1 microm(2)/msec and the mean RA was 24.3 +/- 4.9%. Normal infant cord neural fiber tract morphology was visualized using a mapping of the predominant diffusion tensor eigenvector. Potential clinical applications of line scan DT-MRI of the spinal cord of preterm and term newborns for assessment of spinal cord injury are discussed. J. Magn. Reson. Imaging 2001;13:949-953.
View details for Web of Science ID 000171296500021
View details for PubMedID 11382958
Evaluation and management of children younger than two years old with apparently minor head trauma: Proposed guidelines PEDIATRICS 2001; 107 (5): 983-993
In children <2 years old, minor head trauma (HT) is a common injury that can result in skull fracture and intracranial injury (ICI). These injuries can be difficult to detect in this age group; therefore, many authors recommend a low threshold for radiographic imaging. Currently, no clear guidelines exist regarding the evaluation and management of head-injured infants. We sought to develop guidelines for management based on data and expert opinion that would enable clinicians to identify children with complications of HT and reduce unnecessary imaging procedures. METHODS.References addressing pediatric HT were generated from a computerized database (Medline). The articles were reviewed and evidence tables were compiled. EXPERT PANEL: The multidisciplinary panel was comprised of nine experts in pediatric HT.A modified Delphi technique was used to develop the guidelines. Before the one meeting, panel members reviewed the evidence and formulated answers to specific clinical questions regarding HT in young children. At the meeting, guidelines were formulated based on data and expert consensus.A management strategy was developed that categorizes children into 4 subgroups, based on risk of ICI. Children in the high-risk group should undergo a computed tomography (CT) scan. Those in the intermediate risk group with symptoms of possible ICI should either undergo CT scan or observation. Those in the intermediate risk group with some risk for skull fracture or ICI should undergo CT and/or skull radiographs or observation. Those in the low-risk group require no radiographic imaging.We have developed a guideline for the evaluation of children <2 years old with minor HT. The effect of these guidelines on clinical outcomes and resource utilization should be evaluated.
View details for Web of Science ID 000168411100022
View details for PubMedID 11331675
Microstructural brain development after perinatal cerebral white matter injury assessed by diffusion tensor magnetic resonance imaging PEDIATRICS 2001; 107 (3): 455-460
Brain injury in premature infants is characterized predominantly by perinatally acquired lesions in the cerebral white matter (WM). The impact of such injury on the subsequent development of cerebral WM is not clear. This study uses diffusion tensor magnetic resonance imaging (MRI) to evaluate the effects of cerebral WM injury on subsequent microstructural brain development in different WM areas of the brain.Twenty premature infants (gestational age: 29.1 +/- 1.9 weeks) were studied by conventional MRI within the first 3 weeks of life and again at term, with the addition at the latter time of diffusion tensor MRI. Ten of the preterm infants had cerebral WM injury identified by the early MRI and were matched with 10 premature infants of similar gestational age and neonatal course but with normal neonatal MRI scans. Diffusion tensor MRI at term was acquired in coronal and axial planes and used to determine the apparent diffusion coefficient, a measure of overall restriction to water diffusion, and the relative anisotropy (RA), a measure of preferred directionality of diffusion, in central WM, anterior frontal WM, occipital WM, temporal WM, and the posterior limb of the internal capsule. Diffusion vector maps were generated from the diffusion tensor analysis to define the microstructural architecture of the cerebral WM regions.At term, the diffusion tensor MRI revealed no difference in apparent diffusion coefficient among preterm infants with or without perinatal WM lesions. By contrast, RA, the measure of preferred directionality of diffusion and thereby dependent on development of axonal fibers and oligodendroglia, was 25% lower in central WM, the principal site of the original WM injury. However, RA was unaffected in relatively uninjured WM areas, such as temporal, anterior frontal, and occipital regions. Notably, RA values in the internal capsule, which contains fibers that descend from the injured cerebral WM, were 20% lower in the infants with WM injury versus those without. Diffusion vector maps showed striking alterations in the size, orientation, and organization of fiber tracts in central WM and in those descending to the internal capsule.Perinatal cerebral WM injury seems to have major deleterious effects on subsequent development of fiber tracts both in the cerebral WM and more distally. The ultimate impact of brain injury in the newborn should be considered as a function not only of tissue destruction, but also of impaired subsequent brain development.
View details for Web of Science ID 000167216200016
View details for PubMedID 11230582
Role of flexion-extension radiographs in blunt pediatric cervical spine injury ACADEMIC EMERGENCY MEDICINE 2001; 8 (3): 237-245
To determine whether flexion-extension cervical spine radiography (FECSR) is abnormal in children who have sustained blunt cervical spine injury (CSI) when standard cervical spine radiography (SCSR) demonstrates no acute abnormalities.This was a blinded radiographic review of 129 patients < or = 16 years of age evaluated at an academic pediatric trauma center during July 1990-March 1996. All patients had SCSR (anteroposterior/lateral views) and FECSR performed for a trauma-related event within seven days of injury.Of 46 patients without acute abnormalities on SCSR, one patient (with final clinical diagnosis of "no CSI") had acute abnormalities on FECSR (95% CI = 0.06% to 11.5%). Of 50 patients with isolated loss of lordosis on SCSR, no patient had acute abnormalities on FECSR (95% CI = 0% to 5.8%). The FECSR review revealed no acute abnormalities in 75 of 83 patients (90.4%) with suspicious findings for CSI viewed on SCSR (95% CI = 81.9% to 95.7%). Complications during FECSR were noted in one patient with transient paresthesias (0.8%) (95% CI = 0.02% to 4.2%).In children who underwent acute radiographic evaluation of blunt cervical spine trauma, FECSR was unlikely to be abnormal when no acute abnormality or isolated loss of lordosis was evident on SCSR. In a subset of patients with suspicious findings for occult CSI on SCSR, FECSR was useful in ruling out ligamentous instability in the acute, posttrauma setting.
View details for Web of Science ID 000167345000004
View details for PubMedID 11229945
Proton magnetic spectroscopic imaging of the child's brain: the response of tumors to treatment NEURORADIOLOGY 2001; 43 (2): 169-177
Our aim was to determine and/or predict response to treatment of brain tumors in children using proton magnetic resonance spectro-scopic imaging (MRSI). We studied 24 patients aged 10 months to 24 years, using MRI and point-resolved spectroscopy (PRESS; TR 2000 TE 65 ms) with volume preselection and phase-encoding in two dimensions on a 1.5 T imager. Multiple logistic regression was used to establish independent predictors of active tumor growth. Biologically vital cell metabolites, such as N-acetyl aspartate and choline-containing compounds (Cho), were significantly different between tumor and control tissues (P < 0.001). The eight brain tumors which responded to radiation or chemotherapy, exhibited lower Cho (P = 0.05), higher total creatine (tCr) (P = 0.02) and lower lactate and lipid (L) (P = 0.04) than 16 tumors which were not treated (except by surgery) or did not respond to treatment. The only significant independent predictor of active tumor growth was tCr (P < 0.01). We suggest that tCr is useful in assessing response of brain tumors to treatment.
View details for Web of Science ID 000166910600013
View details for PubMedID 11326567
Neuroimaging and the timing of fetal and neonatal brain injury. Journal of perinatology 2001; 21 (1): 44-60
Current and advanced structural and functional neuroimaging techniques are presented along with guidelines for utilization and principles of imaging diagnosis in fetal and neonatal central nervous system abnormalities. Pattern of injury, timing issues, and differential diagnosis are addressed with emphasis on neurovascular disease. Ultrasonography and computed tomography provide relatively rapid and important screening information regarding gross macrostructural abnormalities. However, current and advanced MRI techniques often provide more definitive macrostructural, microstructural, and functional imaging information.
View details for PubMedID 11268868
Periventricular nodular heterotopia in patients with filamin-1 gene mutations: neuroimaging findings PEDIATRIC RADIOLOGY 2000; 30 (11): 748-755
The filamin-1 (FLN-1) gene is responsible for periventricular nodular heterotopia (PNH), which is an X-linked dominant neuronal migration disorder.To review the clinical and imaging findings in a series of patients with documented filamin-1 mutations.A retrospective review of the medical records and MR studies of a series of patients with PNH and confirmed FLN-1 mutations was done. There were 16 female patients (age range: .67-71 years; mean = 28.6) with filamin-1 gene mutations.In six of the patients the same mutation was inherited in four generations in one pedigree. In a second pedigree, a distinct mutation was found in two patients in two generations. In a third pedigree, a third mutation was found in four patients in two generations. The remaining four patients had sporadic de novo mutations that were not present in the parents. Ten patients had seizures, and all patients had normal intelligence. In all 16 patients MR demonstrated bilateral near-continuous PNH. There were no consistent radiographic or clinical differences between patients carrying different mutations.Patients with confirmed FLN-1 gene mutations are usually female and have a distinctive MR pattern of PNH. Other female patients with this same MR pattern probably harbor FLN-1 mutations and risk transmission to their progeny. This information is important for genetic counseling.
View details for Web of Science ID 000165419100005
View details for PubMedID 11100490
Tuberous sclerosis in the fetus: Second-trimester diagnosis of subependymal tubers with ultrafast MR imaging AMERICAN JOURNAL OF ROENTGENOLOGY 2000; 175 (4): 1067-1069
Prominent basal emissary foramina in syndromic craniosynostosis: Correlation with phenotypic and molecular diagnoses AMERICAN JOURNAL OF NEURORADIOLOGY 2000; 21 (9): 1707-1717
Jugular foraminal stenosis (JFS) or atresia (JFA) with collateral emissary veins (EV) has been documented in syndromic craniosynostosis. Disruption of EV during surgery can produce massive hemorrhage. Our purpose was to describe the prevalence of prominent basal emissary foramina (EF), which transmit enlarged EV, in syndromic craniosynostosis. Our findings were correlated with phenotypic and molecular diagnoses.We reviewed the medical records and imaging examinations of 33 patients with syndromic craniosynostosis and known fibroblast growth factor receptor (FGFR) mutations. All patients underwent CT and 14 MR imaging. The cranial base was assessed for size of occipitomastoid EF and jugular foramina (JF). Vascular imaging studies were available from 12 patients. A control group (n = 76) was used to establish normal size criteria for JF and EF.Phenotypic classification included Crouzon syndrome (n = 10), crouzonoid features with acanthosis nigricans (n = 3), Apert syndrome (n = 10), Pfeiffer syndrome (n = 4), and clinically unclassifiable bilateral coronal synostosis (n = 6). EF > or = 3 mm in diameter and JFS or JFA were identified in 23 patients with various molecular diagnoses. Vascular imaging in patients with JFS or JFA and enlarged EF revealed atresia or stenosis of the jugular veins and enlarged basal EV. JFA was seen in all patients with the FGFR3 mutation with crouzonoid features and acanthosis nigricans. Four patients had prominent EF without JFS. Six patients had normal JF and lacked enlarged EF.Enlarged basal EF are common in syndromic craniosynostosis and are usually associated with JFS or JFA. Bilateral basilar venous atresia is most common in patients with the FGFR3 ala391glu mutation and crouzonoid features with acanthosis nigricans, but may be found in patients with FGFR2 mutations. Skull base vascular imaging should be obtained in patients with syndromic craniosynostosis with enlarged EF.
View details for Web of Science ID 000090110900024
View details for PubMedID 11039354
MR line-scan diffusion imaging of the spinal cord in children AMERICAN JOURNAL OF NEURORADIOLOGY 2000; 21 (7): 1344-1348
Diffusion imaging has been widely used in the brain, but its application in the spinal cord has been limited. Using line-scan diffusion imaging (LSDI), a technique that is less sensitive to magnetic susceptibility and motion artifacts than are other diffusion techniques, we have successfully imaged the spinal cord in children. The apparent diffusion coefficient and relative diffusion anisotropy of the normal spinal cord were measured. LSDI was compared with echo-planar diffusion imaging of the spine in three patients.
View details for Web of Science ID 000165054800032
View details for PubMedID 10954293
Registration of three-dimensional MR and CT studies of the cervical spine AMERICAN JOURNAL OF NEURORADIOLOGY 2000; 21 (2): 282-289
A three-dimensional image registration technique for CT and MR studies of the cervical spine was evaluated for feasibility and efficacy. Registration by means of external fiducial markers was slightly more accurate than registration by anatomic landmarks. The interrelationships between bony (eg, neural foramina) and soft tissue structures (eg, nerve roots) in the cervical spine were more conspicuous on registered images than on conventional displays. Registration of CT and MR images may be used to examine more precisely the relationships between bony and soft tissue structures of the cervical spine.
View details for Web of Science ID 000085398700011
View details for PubMedID 10696009
CT findings in hyperacute nonaccidental brain injury PEDIATRIC RADIOLOGY 2000; 30 (2): 74-81
Central nervous system abnormalities assessed with prenatal magnetic resonance imaging OBSTETRICS AND GYNECOLOGY 1999; 94 (6): 1011-1019
To determine the frequency at which magnetic resonance imaging (MRI) provides additional information in fetuses with suspected central nervous system (CNS) abnormalities on ultrasound.Between May 1, 1996, and March 26, 1999, 83 women with 90 fetuses (including seven sets of live twins) had 91 ultrasonographic and MRI examinations of the fetal CNS. Eight women were studied twice, one for two different indications. If referrals came from outside our institution, a confirmatory sonogram was obtained. Indications for examination were ventriculomegaly (n = 25), suspected neural tube defect (n = 16), arachnoid cyst (n = 12), large cisterna magna (n = 11), and miscellaneous indications (n = 20).Magnetic resonance imaging findings led to changed diagnoses in 26 (40%) of 66 fetuses with abnormal confirmatory sonograms. Magnetic resonance imaging findings not found by ultrasound included partial or complete agenesis of the corpus callosum (n = 11), porencephaly (n = 6), hemorrhage (n = 5), tethered cord (n = 3), cortical gyral abnormality (n = 2), cortical cleft (n = 2), midbrain abnormality (n = 2), and partial or complete agenesis of the septi pellucidi (n = 3), as well as holoprosencephaly, cerebellar hypoplasia, subependymal and cortical tubers, vascular malformation, and vermian cysts (one case each). Abnormalities better delineated by MRI than ultrasound included three cephaloceles, a dural arteriovenous malformation, one distal sacral neural tube defect, and the mass effect of three arachnoid cysts. That information was used to alter patient counseling and at times management.When a CNS anomaly is detected by sonography or suspected on ultrasound, MRI findings might lead to altered diagnosis and patient counseling.
View details for Web of Science ID 000083792800020
View details for PubMedID 10576192
Periventricular white matter injury in the premature infant is followed by reduced cerebral cortical gray matter volume at term ANNALS OF NEUROLOGY 1999; 46 (5): 755-760
Periventricular white matter injury, that is, periventricular leukomalacia (PVL), the dominant form of brain injury in the premature infant, is the major neuropathological substrate associated with the motor and cognitive deficits observed later in such infants. The nature of the relationship of this lesion to the subsequent cognitive deficits is unclear, but such deficits raise the possibility of cerebral cortical neuronal dysfunction. Although cortical neuronal necrosis is not a prominent feature of brain injury in premature infants, the possibility of a deleterious effect of PVL on subsequent cerebral cortical development has not been investigated. An advanced quantitative volumetric three-dimensional magnetic resonance imaging technique was used to measure brain tissue volumes at term in premature infants with earlier ultrasonographic and magnetic resonance imaging evidence of PVL (mean gestational age at birth, 28.7 +/- 2.0 weeks; n = 10), in premature infants with normal imaging studies (mean gestational age at birth, 29.0 +/- 2.1 weeks; n = 10), and in control term infants (n = 14). Premature infants with PVL had a marked reduction in cerebral cortical gray matter at term compared with either premature infants without PVL or normal term infants (mean +/- SD: PVL, 157.5 +/- 41.5 ml; no PVL, 211.7 +/- 25.4 ml; normal term, 218.8 +/- 21.3 ml). As expected, a reduction in the volume of total brain myelinated white matter was also noted (mean +/- SD: PVL, 14.5 +/- 4.6 ml; no PVL, 23.1 +/- 6.9 ml; normal term, 27.6 +/- 10.3 ml). An apparent compensatory increase in total cerebrospinal fluid volume also was found (mean +/- SD: PVL, 64.5 +/- 15.2 ml; no PVL, 52.0 +/- 24.1 ml; normal term, 32.9 +/- 13.5 ml). PVL in the premature infant is shown for the first time to be followed by impaired cerebral cortical development. These findings may provide insight into the anatomical correlate for the intellectual deficits associated with PVL in the premature infant.
View details for Web of Science ID 000083498000011
View details for PubMedID 10553993
Nontuberculous mycobacterial infection of the head and neck in immunocompetent children: CT and MR findings AMERICAN JOURNAL OF NEURORADIOLOGY 1999; 20 (10): 1829-1835
Infections caused by nontuberculous mycobacteria (NTM) commonly manifest as cervicofacial adenitis in otherwise healthy children. The aim of this study was to characterize the imaging findings of NTM infection of the head and neck in immunocompetent children.The medical records and imaging examinations (CT in 10, MR in two) were reviewed in 12 immunocompetent children with NTM infection of the head and neck.The usual presentation (n = 9) was of an enlarging, non-tender mass with violaceous skin discoloration, unresponsive to conventional antibiotics. The duration of symptoms was 6 days to 5 months. Imaging revealed asymmetric adenopathy with contiguous low-density ring-enhancing masses in all patients. There was cutaneous extension in 10 patients. Inflammatory stranding of the subcutaneous fat was minimal (n = 9) or absent (n = 2) in 11 patients. The masses involved the submandibular space (n = 3), the parotid space (n = 2), the cheek (n = 1), the anterior triangle of the neck (n = 2), the submandibular and parotid spaces (n = 2), the parotid space and neck (n = 1), and the neck and retropharyngeal space (n = 1). Surgical management included incision and drainage only (n = 2), incision and drainage with curettage (n = 2), excisional biopsy after incision and drainage (n = 1), excisional biopsy only (n = 5), superficial parotidectomy only (n = 1), and superficial parotidectomy with contralateral excisional biopsy (n = 1). All patients improved in response to surgery and long-term antimycobacterial antibiotics.NTM infection of the head and neck has a characteristic clinical presentation and imaging appearance. Recognition of this disease is important; appropriate treatment is excision and, in selected cases, antimycobacterial therapy.
View details for Web of Science ID 000083887500011
View details for PubMedID 10588104
MR line-scan diffusion-weighted imaging of term neonates with perinatal brain ischemia AMERICAN JOURNAL OF NEURORADIOLOGY 1999; 20 (9): 1658-1670
MR diffusion-weighted imaging provides early demonstration of neonatal brain infarction. The evolution and limitations of diffusion-weighted imaging findings in newborns, however, have not been evaluated. Using line-scan diffusion imaging (LSDI), we investigated perinatal ischemic brain injury.Nineteen term newborns (age, 9 hours to 8 days; mean age, 2.6 days) with perinatal brain ischemia were evaluated using LSDI (1520/62.5/1 [TR/TE/excitations]) (b maximum = 750 s/mm2) and T1- and T2-weighted spin-echo (conventional) MR imaging. Follow-up examinations were performed in seven patients and autopsy in one. Apparent diffusion coefficients (ADCs) were measured in deep gray matter, white matter, the cortex, and focal lesions.Based on conventional MR imaging or pathologic findings, patients were divided into two groups. Group 1 (n = 12) had symmetric/diffuse injury consistent with global hypoperfusion. Group 2 (n = 7) had focal/multifocal injury suggesting cerebrovascular occlusion. ADCs were abnormal at initial examination in 10 newborns in group 1 and in all newborns in group 2. The results of LSDI were abnormal before conventional MR imaging was performed in three newborns in group 1. ADCs were maximally decreased between days 1 and 3 in deep gray matter, perirolandic white matter, and focal lesions. Delayed decreases in ADCs were observed in subcortical white matter from days 4 through 10 in three patients in group 1.After global hypoperfusion, LSDI showed deep gray matter and perirolandic white matter lesions before conventional MR imaging. LSDI may underestimate the extent of injury, however, possibly because of variations in the compartmentalization of edema, selective vulnerability, and delayed cell death. Differences in LSDI of symmetric/diffuse and focal/multifocal lesions may reflect differences in pathophysiology or timing of the injury. These findings may have implications for acute interventions.
View details for Web of Science ID 000083376500017
View details for PubMedID 10543637
Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up PEDIATRIC RADIOLOGY 1999; 29 (9): 662-668
BACKGROUND. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management.A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2-11 years; mean = 5.2 years).Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5-7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2-11 years). There has been one death. The majority of tumors were pilocytic astrocytomas.Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.
View details for Web of Science ID 000082300900003
View details for PubMedID 10460326
An unresponsive infant in the emergency room. Seminars in pediatric neurology 1999; 6 (3): 225-227
The physician must be aware of the computed tomography appearance of an acute-hyperacute subdural hematoma in child abuse and not mistake it for chronic subdural hematoma with "spontaneous" rebleeding. As always, the imaging findings must be correlated with the clinical findings. Clinical and imaging findings of injury out of proportion to the history, and injuries of different ages are the key indicators to the possibility of child abuse, particularly when encountered in a young infant.
View details for PubMedID 10522344
Occult lumbosacral dysraphism in children and young adults: Diagnostic performance of fast screening and conventional MR imaging RADIOLOGY 1999; 211 (3): 767-771
To compare fast screening and conventional magnetic resonance (MR) imaging for the detection of occult dysraphic myelodysplasias in children and young adults.A retrospective case-control study included 101 patients (mean age, 4.9 years; range, 1 day to 26 years) suspected of having occult lumbosacral dysraphism. Sixty case patients had myelodysplastic lesions (19 filar lipoma, 14 syringomyelia, 10 intradural lipoma, eight dermal sinus, five diastematomyelia, five lipomyelomeningocele, two caudal regression syndrome); 41 control patients had no dysraphic lesions; 17 patients had associated renal anomalies. Two neuroradiologists reviewed MR images from conventional and fast screening protocols. Diagnostic performance parameters included sensitivity, specificity, and area under the receiver operating characteristic curve (Az value).The sensitivity of conventional and fast screening MR studies was 97.1% and 98.5%, respectively, the specificity was 90.9% and 84.8%, respectively. The Az value was 0.973 for the fast screening and 0.976 for the conventional MR studies (P = .83). Interobserver agreement was very good for fast screening images (kappa = 0.68) and excellent for conventional images (kappa = 0.75). For renal anomalies, the Az value was 0.786 and 0.853 for fast screening and conventional MR imaging, respectively (P = .28).Conventional three-plane lumbosacral MR imaging in children and young adults suspected of having occult dysraphism provides better diagnostic information than does fast screening two-plane MR imaging because of its higher specificity and interobserver agreement.
View details for Web of Science ID 000080446000027
View details for PubMedID 10352604
The postmigrational development of polymicrogyria documented by magnetic resonance imaging from 31 weeks' postconceptional age ANNALS OF NEUROLOGY 1999; 45 (6): 798-801
We report the case of a 27-week premature infant in whom magnetic resonance imaging (MRI) at 4 postnatal weeks (postconceptional age, 31 weeks), term, and 6 months of age documented the postnatal postmigrational evolution of bilateral perisylvian polymicrogyria. The polymicrogyria was readily detected by ultrafine 1.5-mm coronal slices on three-dimensional, Fourier-transformed, spoiled gradient-recalled and T2-weighted MRI sequences. These MRI sequences provide the first in vivo documentation of the postmigrational evolution of polymicrogyria. The likelihood that the polymicrogyria was related to an ischemic encephaloclastic mechanism is supported by the simultaneous occurrence of periventricular leukomalacia.
View details for Web of Science ID 000080715200016
View details for PubMedID 10360774
Obstetric MR imaging RADIOLOGY 1999; 211 (3): 609-617
The surge in the development of fast magnetic resonance (MR) techniques has revolutionized our ability to image the pregnant patient and the fetus. Fast MR imaging techniques provide excellent resolution for imaging the maternal and fetal anatomies without the need for sedation. This article addresses the use of fast MR imaging techniques in the evaluation of the pregnant patient for adnexal masses, pelvimetry, hydroureteronephrosis of pregnancy, and placenta accreta. In addition, fetal anomalies for which MR imaging has proved useful, such as ventriculomegaly, arachnoid cysts, and abdominal masses, are described.
View details for Web of Science ID 000080446000004
View details for PubMedID 10352581
Early detection of periventricular leukomalacia by diffusion-weighted magnetic resonance imaging techniques JOURNAL OF PEDIATRICS 1999; 134 (5): 631-634
Periventricular leukomalacia (PVL), the principal form of brain injury in the premature infant, is characterized by overt focal necrotic lesions in periventricular white matter and less prominent, more diffuse cerebral white matter injury. The early detection of the latter, diffuse component of PVL is not consistently possible with conventional brain imaging techniques. We demonstrate the early detection of the diffuse component of PVL by diffusion-weighted magnetic resonance imaging (DWI). In a premature infant with no definite cerebral abnormality detectable by cranial ultrasonography or conventional magnetic resonance imaging, DWI showed a striking bilateral decrease in water diffusion in cerebral white matter. The DWI abnormality (ie, decreased apparent diffusion coefficient) was similar to that observed with acute cerebral ischemic lesions in adults. At 10 weeks of age, conventional magnetic resonance imaging and ultrasonography showed striking changes consistent with PVL, including the presence of small cysts. The observations indicate the importance of DWI in the early identification of the diffuse component of PVL and also perhaps the role of ischemia in the pathogenesis of the lesion.
View details for Web of Science ID 000080244600021
View details for PubMedID 10228300
Cortical development and maturation in normal and abnormal fetuses as assessed with prenatal MRI RIVISTA DI NEURORADIOLOGIA 1999; 12: 93-96
MR line scan diffusion imaging of the brain in children AMERICAN JOURNAL OF NEURORADIOLOGY 1999; 20 (3): 419-425
MR imaging of the self-diffusion of water has become increasingly popular for the early detection of cerebral infarction in adults. The purpose of this study was to evaluate MR line scan diffusion imaging (LSDI) of the brain in children.LSDI was performed in four volunteers and 12 patients by using an effective TR/TE of 2736/89.4 and a maximum b value of 450 to 600 s/mm2 applied in the x, y, and z directions. In the volunteers, single-shot echo planar imaging of diffusion (EPID) was also performed. The patients (10 boys and two girls) ranged in age from 2 days to 16 years (average age, 6.6 years). Diagnoses included acute cerebral infarction, seizure disorder, posttraumatic confusion syndrome, complicated migraine, residual astrocytoma, encephalitis, hypoxia without cerebral infarction, cerebral contusion, and conversion disorder. In all patients, routine spin-echo images were also acquired. Trace images and apparent diffusion coefficient maps were produced for each location scanned with LSDI.In the volunteers, LSDI showed less chemical-shift and magnetic-susceptibility artifact and less geometric distortion than did EPID. LSDI was of diagnostic quality in all studies. Diffusion abnormalities were present in five patients. Restricted diffusion was present in the lesions of the three patients with acute cerebral infarction. Mildly increased diffusion was present in the lesions of encephalitis and residual cerebellar astrocytoma. No diffusion abnormalities were seen in the remaining seven children.LSDI is feasible in children, provides high-quality diffusion images with less chemical-shift and magnetic-susceptibility artifact and less geometric distortion than does EPID, and complements the routine MR examination.
View details for Web of Science ID 000079812700011
View details for PubMedID 10219407
Evaluation of fetal central nervous system abnormalities with ultrafast MRI RIVISTA DI NEURORADIOLOGIA 1999; 12: 113-116
Cortical maturation in normal and abnormal fetuses as assessed with prenatal MR imaging RADIOLOGY 1999; 210 (3): 751-758
To establish the appearance of normal fetal cortical development in utero and compare it with the appearance of abnormal cortical development.Magnetic resonance (MR) images of the brain in 53 normal and 40 abnormal fetuses at 14-38 weeks gestational age (GA) were reviewed. The GAs at the time of MR imaging visualization of the fissures or sulci were compared with the GA guidelines based on neuroanatomic studies.In normal fetuses, the sulcation landmarks appeared on MR images in the order predicted by using anatomic studies, with a 0-8-week lag in the MR imaging visualization of the sulci compared with the reported time of visualization of the sulci in anatomic specimens. When landmarks were grouped by range of GAs, the expected MR imaging sulcation landmarks in the group with younger GAs than the actual GA were seen in 50 of 53 (94%) normal fetuses, in five of nine fetuses (56%, P < .05) with isolated mild ventriculomegaly, and in 24 of 31 fetuses (77%, P < .05) with other CNS anomalies.Normal fetal cortical maturation at MR imaging follows a predictable course that is slightly delayed compared with that described in neuroanatomic specimens. This maturation is often further delayed in fetuses with CNS abnormalities.
View details for Web of Science ID 000078796500026
View details for PubMedID 10207478
Fetal CNS anomalies revealed on ultrafast MR imaging AMERICAN JOURNAL OF ROENTGENOLOGY 1999; 172 (3): 813-818
Neuroimaging of scoliosis in childhood NEUROIMAGING CLINICS OF NORTH AMERICA 1999; 9 (1): 195-?
A curvature abnormality may be the initial or major presenting feature in a child with disease of the spinal column or spinal neuraxis. A simplified classification of common spinal curvature abnormalities of childhood include idiopathic, congenital/dysraphic, skeletal dysplasia, neurofibromatosis, and painful. The great majority of childhood scoliosis falls into the idiopathic category. Atypical clinical or radiographic features in a presumed idiopathic scoliosis may indicate an otherwise occult tumor or hydrosyringomyelia, or may be a consequence of increasing curvature with disk protrusion, nerve impingement, or cord attenuation. Neuroimaging beyond plain films is commonly necessary for atypical idiopathic scoliosis and for the other categories of scoliosis listed.
View details for Web of Science ID 000079259200013
View details for PubMedID 9974506
Imaging of neuroendocrine disorders of childhood NEUROIMAGING CLINICS OF NORTH AMERICA 1999; 9 (1): 157-?
This article focuses on the neuroendocrine disorders of childhood. The commonly associated neuroradiologic abnormalities of the hypothalamic-pituitary axis are covered in detail.
View details for Web of Science ID 000079259200011
View details for PubMedID 9974504
Imaging of pediatric temporal bone abnormalities NEUROIMAGING CLINICS OF NORTH AMERICA 1999; 9 (1): 133-?
Disorders involving the temporal bone in children may be associated with considerable morbidity. Hearing loss occurring during childhood may impair cognitive development and should therefore be diagnosed and treated as soon as possible. Thin section high resolution CT and MR imaging of the temporal bone provide critical information about a variety of developmental, inflammatory, traumatic, and neoplastic disorders, and are used for diagnostic purposes and to help determine therapeutic options.
View details for Web of Science ID 000079259200010
View details for PubMedID 9974503
Head and neck vascular anomalies of childhood NEUROIMAGING CLINICS OF NORTH AMERICA 1999; 9 (1): 115-?
Vascular anomalies of the head and neck are common lesions of childhood. The vascular anomalies can be divided into hemangiomas and vascular malformations. Each of these lesions has a characteristic imaging appearance. Correctly classifying the anomaly is essential in directing the treatment of these lesions.
View details for Web of Science ID 000079259200009
View details for PubMedID 9974502
Helical CT angiography: Dynamic cerebrovascular imaging in children AMERICAN JOURNAL OF NEURORADIOLOGY 1999; 20 (2): 328-334
The purpose of this study was to assess the feasibility of helical CT cerebrovascular imaging (CTCVI) in children and to make initial comparisons with MR angiography and digital subtraction angiography (DSA).Twenty-six patients, ages 3 days to 17 years, were examined with CTCVI. Patients were scanned with 1-mm collimation and 2:1 pitch 30 seconds after the initiation of a hand injection of 2 mL/kg nonionic contrast material (320 mg/dL iodine) with a maximum dose that did not exceed 80 mL (minimum volume, 5 mL in a 2.5-kg infant). Reconstructions were done using maximum intensity projection and integral rendering algorithms. Four patients had CTCVI, MR angiography, and DSA (42 vessels studied) and nine patients had CTCVI and DSA (136 vessels studied). Scores of 1 (not present) to 3 (present in continuity to the first bifurcation) were assigned independently by two radiologists to 32 vessels in each correlated case for each available technique.There were no technical failures. CTCVI depicted 18 thrombosed dural sinuses, three vascular malformations, one intracranial aneurysm, and four tumors. Ninety-five percent of the vessels seen with DSA were also seen with CTCVI. CTCVI identified all vessels seen on MR angiography.Helical CTCVI is an effective technique for assessing the intracranial circulation in children. In this initial comparison, CTCVI showed more vascular detail than MR angiography, and had fewer technical limitations.
View details for Web of Science ID 000078982400034
View details for PubMedID 10094365
Neurologic complications of cerebral angiography in childhood moyamoya syndrome PEDIATRIC RADIOLOGY 1998; 28 (11): 824-829
Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders.
View details for Web of Science ID 000077029100005
View details for PubMedID 9799310
Microstructural development of human newborn cerebral white matter assessed in vivo by diffusion tensor magnetic resonance imaging PEDIATRIC RESEARCH 1998; 44 (4): 584-590
Alterations of the architecture of cerebral white matter in the developing human brain can affect cortical development and result in functional disabilities. A line scan diffusion-weighted magnetic resonance imaging (MRI) sequence with diffusion tensor analysis was applied to measure the apparent diffusion coefficient, to calculate relative anisotropy, and to delineate three-dimensional fiber architecture in cerebral white matter in preterm (n = 17) and full-term infants (n = 7). To assess effects of prematurity on cerebral white matter development, early gestation preterm infants (n = 10) were studied a second time at term. In the central white matter the mean apparent diffusion coefficient at 28 wk was high, 1.8 microm2/ms, and decreased toward term to 1.2 microm2/ms. In the posterior limb of the internal capsule, the mean apparent diffusion coefficients at both times were similar (1.2 versus 1.1 microm2/ms). Relative anisotropy was higher the closer birth was to term with greater absolute values in the internal capsule than in the central white matter. Preterm infants at term showed higher mean diffusion coefficients in the central white matter (1.4 +/- 0.24 versus 1.15 +/- 0.09 microm2/ms, p = 0.016) and lower relative anisotropy in both areas compared with full-term infants (white matter, 10.9 +/- 0.6 versus 22.9 +/- 3.0%, p = 0.001; internal capsule, 24.0 +/- 4.44 versus 33.1 +/- 0.6% p = 0.006). Nonmyelinated fibers in the corpus callosum were visible by diffusion tensor MRI as early as 28 wk; full-term and preterm infants at term showed marked differences in white matter fiber organization. The data indicate that quantitative assessment of water diffusion by diffusion tensor MRI provides insight into microstructural development in cerebral white matter in living infants.
View details for Web of Science ID 000076148500019
View details for PubMedID 9773850
Cyclosporin A acute encephalopathy and seizure syndrome in childhood: Clinical features and risk of seizure recurrence JOURNAL OF CHILD NEUROLOGY 1998; 13 (7): 336-344
Cyclosporin A is associated with an acute encephalopathy including seizures and alterations in mental status, herein referred to as cyclosporin A acute encephalopathy and seizure syndrome. The clinical history, electroencephalogram (EEG), and neuroimaging findings in 19 children with cyclosporin A acute encephalopathy and seizure syndrome over a 10-year period were reviewed in order to delineate clinical characteristics, imaging features, and to determine the risk of seizure recurrence in this population. All 19 had motor seizures associated with other features of cortical and subcortical dysfunction. The acute mean cyclosporin A level was 342 microg/L, but was within the "therapeutic" range in five cases. Brain imaging by computed tomography (CT) or magnetic resonance imaging (MRI) in the acute or subacute phase revealed lesions characteristic of cyclosporin A toxicity in 14 cases. Acute EEG abnormalities were present in all and included epileptiform discharges or focal slowing. Patients were followed for a median of 49 months (1-9 years). Follow-up imaging (n = 10) showed lesion resolution or improvement in the majority while EEG (n = 10) had normalized in only three. Seizures recurred in six patients and only in those with persistent EEG or imaging abnormalities. No patient had a second episode of cyclosporin A associated neurotoxicity or seizure. It appears that a significant risk of seizure recurrence exists following cyclosporin A acute encephalopathy and seizure syndrome and primarily in those children with persistent EEG or imaging abnormalities.
View details for Web of Science ID 000075150900006
View details for PubMedID 9701483
Tectal tumors of childhood: Clinical and imaging follow-up AMERICAN JOURNAL OF NEURORADIOLOGY 1998; 19 (5): 977-983
This study was done to determine which clinical and imaging findings best correlate with outcome in children with tectal tumors.A retrospective review was done of the medical records and imaging studies of 32 children (16 boys and 16 girls; mean age, 8 years) with tectal tumors. Eight children had CT, 11 had MR imaging, and 13 had both CT and MR studies. Findings from surgical and pathologic reports as well as from follow-up examinations (mean follow-up period, 5 years; range, 3.6 months to 17 years) were included in the review.All patients had hydrocephalus and all but one required CSF diversion. The tectum was the center of the tumor in all cases and the majority of the tumors appeared isodense on CT scans, isointense on T1-weighted MR images, and hyperintense on T2-weighted images. Twenty patients required no further treatment. In this group, the mean maximum tumor diameter was 1.8 cm and enhancement occurred in two cases. At follow-up, 18 patients had stable tumor size, one had an increase in tumor size with cyst formation but no worsening of symptoms, and one had a decrease in tumor size. Twelve patients required further treatment (excision and/or radiotherapy) because of progression as indicated by either increased tumor size or worsening of symptoms. In this group, the mean maximum tumor diameter was 2.5 cm and contrast enhancement occurred in nine cases. Further follow-up in this group showed decreased tumor size in eight and stable residual tumor in three.Tectal tumors in childhood have variable behavior. MR imaging assists in the clinical determination of which children need treatment beyond CSF diversion. Larger tumor size and enhancement are radiologic predictors of the need for further treatment.
View details for Web of Science ID 000073588000036
View details for PubMedID 9613524
Cerebellar epilepsy NEUROSURGERY 1998; 42 (5): 1106-1111
Relation of seizures after cardiac surgery in early infancy to neurodevelopmental outcome CIRCULATION 1998; 97 (8): 773-779
The outcome of infants who have transient seizures after open heart surgery has not been studied. Using the database of the Boston Circulatory Arrest Study involving 171 children with D-transposition of the great arteries, we explored the relationship between early postoperative clinical and EEG seizures and neurodevelopmental outcomes at ages 1 and 2 1/2 years.At 1 year, children returned for developmental and neurological evaluations and MRI. Parent-completed developmental questionnaires were collected at 2 1/2 years of age. At 1 year, children with early postoperative seizures had lower Psychomotor Development Index (motor function) scores (clinical seizures: 12.9 mean difference [MD]; 95% confidence interval [CI], 2.2 to 23.6; P=.02; EEG seizures: 13.3 MD; 95% CI, 6.8 to 19.7; P<.001). Mental Developmental Index scores of children with clinical or EEG seizures were also lower, but the differences were not statistically significant. Infants with seizures were more likely to have an abnormal neurological examination (clinical seizures: 78% versus 31%; P=.008; EEG seizures: 58% versus 34%; P=.04). Children with EEG seizures were more likely to have MRI abnormalities (43% versus 13%, P=.002). At age 2 1/2, children with EEG seizures had lower scores in several areas of function.In infants undergoing the arterial switch operation for correction of D-transposition of the great arteries, transient postoperative clinical and EEG seizures were associated with worse neurodevelopmental outcomes at ages 1 and 2 1/2 years as well as neurological and MRI abnormalities at 1 year of age. The occurrence of such seizures may provide an early sign of brain injury with neurological and developmental sequelae.
View details for Web of Science ID 000072200800011
View details for PubMedID 9498541
Efficacy of fast screening MR in children and adolescents with suspected intracranial tumors AMERICAN JOURNAL OF NEURORADIOLOGY 1998; 19 (3): 529-534
Our purpose was to determine the sensitivity, specificity, and receiver operator characteristic (ROC) curve of a fast screening MR protocol in children and adolescents with suspected intracranial tumors.One hundred forty-one patients (mean age, 9.7 years; range, 2 months to 23.5 years) with suspected brain tumor were entered in a case-control study. Eighty-seven patients had intracranial tumors (31 suprasellar/hypothalamic, 27 supratentorial, 26 infratentorial, and three pineal) and 54 patients in the control group had other disorders. Two neuroradiologists reviewed blindly a detailed three-sequence conventional protocol (acquisition time, 8 minutes 27 seconds) and a two-sequence fast screening MR protocol (acquisition time, 4 minutes 44 seconds).Sensitivity and specificity of the fast screening protocol for intracranial tumors was 100% and 92.6%, respectively. The areas under the ROC curves were 0.966 for the fast screening and 0.980 for the conventional MR protocol. No diagnostic performance difference was found between the ROC curves using the Az index. A kappa statistic of .93 for both examinations indicated excellent interobserver agreement. Additional MR sequences and other neuroimaging studies were not deemed necessary to exclude the presence of an intracranial tumor.A fast dual-plane brain MR protocol may be adequate to screen children and adolescents thought to have an intracranial tumor. The less than 5 minute acquisition time allows a complete examination (including preparation) to be performed in 10 to 15 minutes. Future studies are recommended before this time-efficient neuroimaging examination is incorporated into clinical practice.
View details for Web of Science ID 000072548100026
View details for PubMedID 9541313
Spastic diplegia as a complication of interferon Alfa-2a treatment of hemangiomas of infancy JOURNAL OF PEDIATRICS 1998; 132 (3): 527-530
We report the development of spastic diplegia in infants during the course of interferon Alfa-2a (IFN) therapy for potentially life-endangering hemangiomas. Five infants who displayed diplegia were selected from a group of 26 infants treated with IFN. Diplegia persisted in three infants, and in the remaining two significant recovery occurred after IFN was discontinued. Magnetic resonance imaging showed no significant brain or spinal abnormalities except minor to moderate delayed myelination in two patients. Myelination was normal on subsequent radiographic examination in all five patients. We conclude that IFN can adversely affect the immature central nervous system and produce spastic diplegia, which is potentially reversible. We recommend careful clinical assessment of neurodevelopmental status during IFN therapy.
View details for Web of Science ID 000072877800034
View details for PubMedID 9544915
Quantitative magnetic resonance imaging of brain development in premature and mature newborns ANNALS OF NEUROLOGY 1998; 43 (2): 224-235
Definition in the living premature infant of the anatomical and temporal characteristics of development of critical brain structures is crucial for insight into the time of greatest vulnerability of such brain structures. We used three-dimensional magnetic resonance imaging (3D MRI) and image-processing algorithms to quantitate total brain volume and total volumes of cerebral gray matter (GM), unmyelinated white matter (WM), myelinated WM, and cerebrospinal fluid (CSF) in 78 premature and mature newborns (postconceptional age, 29-41 weeks). Total brain tissue volume was shown to increase linearly at a rate of 22 ml/wk. Total GM showed a linear increase in relative intracranial volume of approximately 1.4% or 15 ml in absolute volume per week. The pronounced increase in total GM reflected primarily a fourfold increase in cortical GM. Unmyelinated WM was found to be the most prominent brain tissue class in the preterm infant younger than 36 weeks of postconceptional age. Although minimal myelinated WM was present in the preterm infant at 29 weeks, between 35 and 41 weeks an abrupt fivefold increase in absolute volume of myelinated WM was documented. Extracerebral and intraventricular CSF was readily quantitated by this technique and found to change minimally. The application of 3D MRI and tissue segmentation to the study of human infant brain from 29 to 41 weeks of postconceptional age has provided new insights into cerebral cortical development and myelination and has for the first time provided means of quantitative assessment in vivo of early human brain development.
View details for Web of Science ID 000071945200012
View details for PubMedID 9485064
Fetal fast MR imaging: Reproducibility, technical quality, and conspicuity of anatomy RADIOLOGY 1998; 206 (2): 549-554
To evaluate the normal appearance of fetal anatomy, the conspicuity of fetal organs, the reproducibility of images, and the limitations to image quality with the use of half-Fourier, single-shot rapid acquisition with relaxation enhancement (RARE) magnetic resonance (MR) imaging.Fifty-four fetuses of 49 pregnancies underwent MR imaging with the half-Fourier, single-shot RARE technique. Two reviewers attempted to identify 47 organs and anatomic regions in each fetus. Organ or region conspicuity, image quality, and the limitations of image quality were graded.Fetal anatomy was well depicted in fetuses over 20 weeks in gestational age. Fetal imaging was limited by gestational age of 20 weeks or less usually owing to the small size of the organ or region being evaluated and, less frequently, by motion.Half-Fourier, single-shot RARE MR imaging provided a detailed and reproducible evaluation of normal fetal anatomy, which can be used as a standard of reference in MR imaging of fetal anomalies.
View details for Web of Science ID 000071627700036
View details for PubMedID 9457211
Imaging of the neonatal central nervous system NEUROSURGERY CLINICS OF NORTH AMERICA 1998; 9 (1): 17-?
This article presents some of the important technical and clinical evaluation of the immature and developing central nervous system. Emphasis is given to ultrasonography, computed tomography, and magnetic resonance imaging. It is our hope that these principles will assist in the evaluation of the neonate and young infant with neurologic problems requiring neurosurgical consultation and intervention.
View details for Web of Science ID 000071880200004
View details for PubMedID 9405763
Childhood optic chiasm gliomas: Radiographic response following radiotherapy and long-term clinical outcome INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 1997; 39 (3): 579-587
In children with chiasmal gliomas, radiation therapy can arrest progressive visual and neurologic impairment. We examined the radiographic response and clinical outcomes after irradiation.Forty-two children (median age at diagnosis, 6.6 years) with chiasmal gliomas were managed as follows: 11 asymptomatic patients with neurofibromatosis-1 (NF-1) were observed only; 2 patients, less than 3 years old, underwent surgery and chemotherapy to delay irradiation; and 29 patients with progressive disease received radiation with or without prior surgery or chemotherapy. Time to radiographic response, long-term tumor control and late sequelae were reviewed for the 29 irradiated patients.The probability of at least 50% radiographic response at 24 months after irradiation was 18.1% and increased to 38.2% by 48 months and 45.9% by 60 months. By actuarial analysis, the median time for such radiographic response was 62 months. For the 29 irradiated patients, the 10-year freedom from progression and overall survival rates were 100% and 89%, respectively (median follow-up for surviving patients, 108 months). Stabilization or improvement in vision occurred in 81% of 26 evaluable irradiated patients.Notable radiographic response may be observed years after irradiation. Radiation therapy provides excellent long-term tumor control and vision preservation or improvement in the majority of patients with progressive chiasmal gliomas.
View details for Web of Science ID A1997YA91700009
View details for PubMedID 9336136
Radiologic-pathologic conference of Children's Hospital Boston: Pineal region mass in a neonate PEDIATRIC RADIOLOGY 1997; 27 (10): 829-831
Prenatal ultrasound examination of a fetus revealed a pineal region mass. Shortly after birth the baby was evaluated with MRI and ultrasound of the brain. The clinical history and the MRI, operative, and histological findings are presented and discussed.
View details for Web of Science ID A1997YC03300019
View details for PubMedID 9323255
Childhood moyamoya disease: hemodynamic MRI PEDIATRIC RADIOLOGY 1997; 27 (9): 727-735
Childhood moyamoya disease is a rare progressive cerebrovascular disease.To evaluate cerebral hemodynamics using dynamic Gd-DTPA-enhanced imaging in children with moyamoya disease.Eight children (2-11 years of age) with the clinical and angiographic findings typical of moyamoya disease, before and/or after surgical intervention (pial synangiosis), underwent conventional MR imaging (MRI) and hemodynamic MR imaging (HMRI). HMRI used a spoiled gradient-echo with low flip angle (10 deg) and long TE (TR/TE = 24/15 ms) to minimize T 1 effects and emphasize T 2* weighting. Raw and calculated hemodynamic images were reviewed. Three-dimensional time-of-flight MR angiography (MRA) and perfusion brain single photon emission computed tomography (SPECT) were also performed.Abnormal hemodynamic maps resulting from vascular stenosis or occlusion and basal collaterals were observed in six patient studies. HMRI depicted perfusion dynamics of affected cerebrovascular territories, detected cortical perfusion deficits, and complemented conventional MRI and MRA. HMRI findings were consistent with those of catheter angiography and perfusion SPECT.Our preliminary experience suggests that HMRI may be of value in the preoperative and postoperative evaluation of surgical interventions in moyamoya disease.
View details for Web of Science ID A1997XX55400001
View details for PubMedID 9285733
Posterior fossa abscess complicating occipital dermal sinus in children INTERNATIONAL JOURNAL OF NEURORADIOLOGY 1997; 3 (5): 435-440
Diencephalic syndrome: Clinical features and imaging findings AMERICAN JOURNAL OF NEURORADIOLOGY 1997; 18 (8): 1499-1505
To emphasize the importance of imaging in children with diencephalic syndrome due to hypothalamic/chiasmatic astrocytomas.Findings in nine patients (mean age, 26 months) with diencephalic syndrome and hypothalamic/chiasmatic astrocytomas were analyzed retrospectively, including reviewing clinical records, imaging examinations, and follow-up studies.Symptoms and signs included failure to thrive (n = 9), nystagmus (n = 3), visual field defects (n = 1), optic pallor (n = 1), emesis (n = 2), and headache (n = 1). All patients had hypothalamic/chiasmatic masses. Five patients underwent biopsy, and, in all cases, specimens showed low-grade astrocytoma. Imaging studies were available in eight patients. All tumors were large (median maximum diameter, 3.5 cm), involved the chiasm and hypothalamus, and showed homogeneous enhancement. Three patients had hydrocephalus and two had metastases. At follow-up, five patients had recurrent disease and two had died.Diencephalic syndrome is a rare cause of failure to thrive in childhood, and diagnosis of a hypothalamic/ chiasmatic astrocytoma might therefore be delayed. The astrocytomas associated with this syndrome are larger, occur at a younger age, and are often more aggressive than other astrocytomas arising in this region.
View details for Web of Science ID A1997XV50000015
View details for PubMedID 9296191
Fetal central nervous system anomalies: MR imaging augments sonographic diagnosis RADIOLOGY 1997; 204 (3): 635-642
To evaluate fetuses with sonographically suspected central nervous system anomalies to determine the frequency with which obstetric magnetic resonance (MR) imaging adds clinically useful information to that provided by ultrasonography (US).US and MR images and diagnoses in 18 pregnant women were reviewed and compared by two radiologists. Postnatal physical examination and imaging findings and fetal autopsy results were standards. Referring physicians were questioned as to how the additional information provided by MR imaging changed patient counseling.In 10 (55%) patients, MR imaging demonstrated 11 additional findings. These findings were agenesis of the corpus callosum (n = 4), cerebellar hypoplasia (n = 2), cortical cleft (n = 2), polymicrogyria (n = 1), porencephaly (n = 1), and partial agenesis of the septi pellucidi (n = 1). In seven (39%) patients, additional information provided by MR imaging altered counseling. In one case of suspected agenesis of the corpus callosum, diagnosis at MR imaging was at least partially incorrect.US and MR imaging are complementary imaging methods in the evaluation of high-risk pregnancy. When a central nervous system anomaly is suspected at US, MR imaging may demonstrate additional findings that can alter patient counseling.
View details for Web of Science ID A1997XR60200009
View details for PubMedID 9280237
Magnetic resonance techniques in the evaluation of the newborn brain CLINICS IN PERINATOLOGY 1997; 24 (3): 693-?
MR imaging provides unequaled sensitivity as compared with US or CT scanning for evaluating developmental changes and pathologic processes in the newborn brain. Myelination can be assessed qualitatively and quantitatively using newer 3D-MR imaging methods. MR imaging provides a much clearer delineation of many developmental disorders, including anomalies of migration and organization, as well as a variety of metabolic disorders and congenital infections. Neonatal intracranial hemorrhage is detected in all its locations by MR imaging. The timing of the hemorrhage is a unique feature of MR imaging. Venous thrombosis also can be identified by MR imaging and confirmed with MR angiography. HIE is the major cause of potentially preventable or reversible brain injury that results in considerable long-term neurologic morbidity. Early detection is crucial for interventions aimed at preventing or reversing ongoing injury. DWI can show early changes at the cellular level that are not detectable by any other imaging modality. MR spectroscopy has further opened the possibility of studying the metabolic mechanisms that define the pathophysiologic events taking place in neonatal brain injury. Both 31P-MR spectroscopy, as a marker of the acute changes in energy metabolism, and 1H-MR spectroscopy, with the measurement of lactate and the excitotoxic aminoacids glutamate and glutamine, have enabled us to study the early and late effects of insults to the newborn brain in a noninvasive fashion. Studies performed to determine the predictive value of MR spectroscopy for later neurodevelopmental outcome after HIE have shown promising results but need further evaluation on larger patient samples. The potential use of these methods in the evaluation of early neuroprotective treatment regimens in the newborn remains to be determined.
View details for Web of Science ID A1997XW36200010
View details for PubMedID 9394867
Optic chiasm astrocytomas of childhood 1. Long-term follow-up PEDIATRIC NEUROSURGERY 1997; 27 (3): 121-128
We reviewed 61 patients seen from 1975 to 1994 with a biopsy-confirmed optic chiasm astrocytoma or a characteristic clinical and imaging presentation. The mean age at diagnosis was 72 months, including 30 who were less than 5 years old. The mean tumor diameter at presentation was 31 mm among 54 tumors measured. Tumors involved the optic nerve in 41, hypothalamus in 38, optic tract in 15, and optic radiations in 2. Four patients, all with neurofibromatosis, received no treatment. Forty patients received conventional radiation therapy at a mean age of 89 months. Chemotherapy was given to 19 children and 7 of these were followed for greater than 1 year. Six of these 7 had progressive disease which required subsequent surgery and/or radiation therapy. Thirty-six patients underwent surgery. We restricted our analysis of outcome to those 38 patients followed for longer than 60 months. Development was normal in 13%, mildly disabled in 53%, severely disabled in 21% and dead in 13%. Children were severely disabled or dead in 47% if younger than 5 years at diagnosis, and 21% if older. There was no endocrine dysfunction in 18% and new endocrine failure was seen in 61%. More posterior tumors did not portend a poorer prognosis. Children less than 5 years old had a poorer prognosis. Neurofibromatosis did not have a marked effect on outcome. Intracranial hypertension was an indicator of a poor prognosis. No specific treatment modality was clearly superior in terms of long-term survival or improvement in quality of life.
View details for Web of Science ID 000072561300002
View details for PubMedID 9548522
Radiologic-Pathologic Conference of Children's Hospital Boston: Intraconal mass in the orbit of an infant PEDIATRIC RADIOLOGY 1997; 27 (8): 682-684
A 16-month-old boy presented with left exophthalmos. He was found to have an enhancing intraconal soft-tissue mass. The differential diagnosis of the mass is discussed. The lesion was proven to be a malignant ectomesenchymoma, a very unusual tumor.
View details for Web of Science ID A1997XZ33800011
View details for PubMedID 9252435
Angiographic changes after pial synangiosis in childhood moyamoya disease AMERICAN JOURNAL OF NEURORADIOLOGY 1997; 18 (5): 837-845
To describe the angiographic changes accompanying the surgical treatment of moyamoya disease by pial synangiosis and to compare these changes with patient outcome.The preoperative and postoperative cerebral angiograms, MR images, and clinical records of 13 children treated with pial synangiosis for moyamoya disease were reviewed.After synangiosis, 10 patients had significant neurologic improvement and three had minimal or no improvement. Postoperative MR images showed no new infarctions. Well-developed (grade A or B) transpial or transdural collaterals to the brain were present at the site of synangiosis in 84% of the surgically treated hemispheres. Cerebrovascular occlusive changes increased postoperatively in 76% of hemispheres. After synangiosis, moyamoya collaterals were increased in 48%, unchanged in 16%, and decreased in 36% of surgically treated hemispheres. All 10 patients with grade A or B collaterals bilaterally after synangiosis were asymptomatic or improved on follow-up.Pial synangiosis typically results in an increase in collaterals from the superficial temporal artery or middle meningeal artery to the brain. Synangiosis appears to result in stabilization or improvement in neurologic symptoms but does not prevent the angiographic progression of disease or the development of moyamoya collaterals. The angiographic demonstration of well-formed collaterals after synangiosis is associated with a favorable clinical outcome.
View details for Web of Science ID A1997WZ04600005
View details for PubMedID 9159360
Brain SPECT evaluation of cerebral perfusion in hemimegalencephaly CLINICAL NUCLEAR MEDICINE 1997; 22 (4): 250-252
Children with headache: Clinical predictors of surgical space-occupying lesions and the role of neuroimaging RADIOLOGY 1997; 202 (3): 819-824
To determine clinical predictors useful in differentiation of surgical lesions from medically treated disorders and the role of neuroimaging in children with headache. MATERIALS' AND METHODS: In a 4-year retrospective study, 315 patients with headache and no known neurologic disorder underwent brain magnetic resonance (MR) imaging. Sixty-nine patients also underwent brain computed tomography (CT). Clinical data were correlated with findings from MR imaging and CT and the final diagnosis by means of logistic regression.Thirteen (4%) patients had surgical space-occupying lesions. Seven independent multivariate predictors of a surgical lesion were identified. Sleep-related headache and no family history of migraine were the strongest predictors. Other predictors included vomiting, absence of visual symptoms, headache of less than 6 months duration, confusion, and abnormal neurologic examination findings. A positive correlation between number of predictors and risk of surgical lesion was noted (P < .0001). No difference between MR imaging and CT was noted in detection of surgical space-occupying lesions, and there were no false-positive or false-negative surgical lesions detected with either modality on the basis of clinical follow-up.Children at high risk on the basis of these criteria usually require neuroimaging, while children at low risk may be safely followed up clinically without neuroimaging.
View details for Web of Science ID A1997WJ45600041
View details for PubMedID 9051039
Database prescan: A time-efficient alternative to brain MRI autoprescan JOURNAL OF MAGNETIC RESONANCE IMAGING 1997; 7 (2): 442-446
The purpose of this study was to determine the feasibility of database prescan as an alternative to conventional autoprescan in pediatric brain MRI. Autoprescan parameters [receiver levels and transmit gain (TG)] were analyzed prospectively in 236 pediatric brain MRI studies. Paired t test and linear regression analysis were performed to determine predictability of autoprescan parameters by database-generated parameters. Signal-to-noise ratio, image quality, and potential time efficiency of database-generated parameters were assessed. No statistical difference (P = .13) and a high correlation between the TG of the axial fast spin echo (FSE) proton density (PD) and axial FSE T2-weighted sequences (r = .92) was seen. Strong correlations were noted between the TG of the sagittal T1-weighted and the TG of the axial FSE PD (r = .79), axial FSE T2-weighted (r = .81), and contrast-enhanced T1-weighted (r = .78) sequences. The receiver levels did not change significantly between sequences. Quantitative and qualitative analyses revealed no differences in the signal-to-noise ratios of the autoprescan and the database-predicted prescan parameters. Implementation of database prescan could improve time efficiency by 28 to 33%. Autoprescan parameters can be predicted by using database-generated information while preserving the diagnostic image quality of the study. Incorporation of database prescan into commercial MRI systems could improve MRI time efficiency and patient throughput.
View details for Web of Science ID A1997WP11800032
View details for PubMedID 9090606
Multivoxel proton MR spectroscopy and hemodynamic MR imaging of childhood brain tumors: Preliminary observations AMERICAN JOURNAL OF NEURORADIOLOGY 1997; 18 (2): 203-218
To assess multivoxel proton MR spectroscopy combined with MR imaging and hemodynamic MR imaging in the evaluation of brain tumors in children and young adults.Fifteen patients with brain tumors and 10 healthy children underwent MR imaging and MR spectroscopy on a 1.5-T system. Ten patients with tumors had both MR spectroscopy and hemodynamic MR imaging. MR spectroscopy data sets with 1 cm3 to 3.4 cm3 resolution were acquired within 8.5 minutes by using a point-resolved spectroscopic, chemical-shift imaging technique in two dimensions with volume preselection. MR imaging was performed using fast spin-echo techniques. Hemodynamic MR imaging data were acquired every 2.5 seconds at one anatomic level using a spoiled gradient-echo sequence during intravenous bolus administration of contrast material.Assessment with multivoxel MR spectroscopy and hemodynamic MR imaging added about 30 minutes to the total MR examination time. Normal tissue exhibited spectral peaks from biologically significant compounds such as N-acetylaspartate (NAA), choline-containing compounds (Cho), and total creatine (tCr). Twelve biopsy-proved tumors exhibited prominent Cho, reduced NAA, variable tCr, and/or lactate or lipids, and two showed increased hemodynamic parameters. Three of the tumors treated with radiation did not reveal prominent levels of Cho. Tissue necrosis had no Cho, NAA, or tCr, and reduced hemodynamics.Preliminary findings by MR spectroscopy combined with MR imaging and hemodynamic MR imaging suggest that regions of active tumor may be differentiated from areas of normal tissue and areas of necrosis. These findings may enable metabolic and hemodynamic characterization of childhood brain tumors as well as suggest their response to therapy.
View details for Web of Science ID A1997WJ80700003
View details for PubMedID 9111654
Jugular foraminal stenosis in Crouzon syndrome PEDIATRIC NEUROSURGERY 1996; 25 (5): 252-255
We describe a 4-year-old boy with Crouzon syndrome, with associated acanthosis nigricans and Chiari-I malformation, who developed increased intracranial pressure necessitating posterior cranial expansion. Postoperatively, an arteriovenous fistula appeared over the mastoid region. Cerebral angiography demonstrated bilateral atresia of the jugular veins and occlusion of the left sigmoid and right transverse sinuses. We propose that increased intracranial venous pressure, secondary to bilateral jugular foraminal stenosis, caused hydrocephaly and venous dilation in the scalp vasculature. The latter set the stage for a traumatic arteriovenous fistula of the scalp, probably resulting from laceration of an adjacent artery and vein. Jugular atresia is a basilar malformation common to achondroplasia and certain eponymous syndromic craniosynostoses. Our patient has a mutation in fibroblast growth factor receptor 3, a different locus in the same gene mutated in achondroplasia.
View details for Web of Science ID A1996XU30900007
View details for PubMedID 9309789
Hemorrhagic pituitary adenomas of adolescence AMERICAN JOURNAL OF NEURORADIOLOGY 1996; 17 (10): 1907-1912
To review the clinical and MR imaging findings in adolescents with hemorrhagic pituitary adenomas and to compare those findings with pathologic results and outcome.We reviewed the clinical records, imaging examinations, surgical and pathologic findings, and follow-up studies in 11 girls and six boys (12 to 20 years old; mean age, 16 years) with pituitary adenomas who were treated at our institution between August 1986 and June 1995.Of the 17 adenomas, eight were macroadenomas (> 1 cm) in patients 14 to 18 years old (three girls, five boys). Six of the macroadenomas were grossly hemorrhagic, and appeared as high-intensity intrasellar/suprasellar masses on all MR sequences obtained before definitive diagnosis and treatment. Clinical presentation in the patients with the hemorrhagic macroadenomas included headache (five), visual field deficits (three), and neuroendocrine symptoms (three). One patient was asymptomatic. The preliminary clinical and imaging diagnoses were craniopharyngioma or Rathke's cyst in five of the six cases. Pathologic diagnoses were prolactinoma in four patients, plurihormonal (prolactin/follicle-stimulating hormone) tumor in one patient, and nonfunctioning adenoma in one patient. Surgical resection was performed in all six hemorrhagic tumors and radiation therapy was required in three cases.Pituitary adenomas uncommonly occur in childhood and are usually seen in adolescence. The majority of the macroadenomas are hemorrhagic and often occur in male subjects. The clinical and MR imaging features may mimic craniopharyngioma or Rathke's cyst. These tumors often require surgery and/or radiation therapy.
View details for Web of Science ID A1996VU08000016
View details for PubMedID 8933876
Magnetic resonance imaging changes after stereotactic radiation therapy for childhood low grade astrocytoma CANCER 1996; 78 (4): 864-873
Stereotactic radiotherapy (SRT) is fractionated radiotherapy delivered under stereotactic guidance to produce highly focal and precise therapy. We studied the incidence of imaging changes that can mimic tumor progression after completion of SRT for childhood low grade astrocytoma.Between June 1992 and September 1994, we prospectively treated 28 children with low grade astrocytomas with SRT. The patients ranged in age from 2 to 22 years (median: 10 yrs) and none had received prior radiation therapy or radiosurgery. Routine fractionation was employed (180-200 centigray[cGy]) to a total dose of 5220-6000 cGy over 5 to 6 weeks. All of the patients underwent initial and follow-up magnetic resonance imaging (MRI) according to protocol.Median clinical follow-up for the 28 patients was 24 months (range, 5-32 mos) with a median radiographic follow-up of 15 months (range, 3-26 mos). Fifteen patients had reduction in tumor size, one patient had stable disease. Twelve patients (43%) developed increased size of the lesion, increased signal intensity or enhancement, cysts or cavitations, and an increase in edema or mass effect on follow-up MRI. Most of these changes occurred between 9 and 12 months after the start of SRT and resolved or decreased by 15 to 21 months. All but one patient had normal or stable neurologic examinations.Treatment-related MRI changes are common after conventionally fractionated schedules using stereotactic radiation techniques for patients with low grade astrocytomas. These changes can be distinguished from tumor progression by their transient nature as well as the general absence of clinical symptoms.
View details for Web of Science ID A1996VA43700025
View details for PubMedID 8756383
Angiography and the evaluation of cerebrovascular disease in childhood NEUROIMAGING CLINICS OF NORTH AMERICA 1996; 6 (3): 561-?
This article outlines indications and techniques for and findings on angiography in pediatric cerebrovascular disease. The clinical entities described and illustrated include extracranial and intracranial pediatric vascular anomalies (hemangiomas and vascular malformations), pediatric aneurysms and occlusive cerebrovascular disease. Features specific to or more common in children are emphasized.
View details for Web of Science ID A1996VE67100003
View details for PubMedID 8873093
Ictal and interictal technetium-99m-bicisate brain SPECT in children with refractory epilepsy JOURNAL OF NUCLEAR MEDICINE 1996; 37 (7): 1101-1106
Identification of epileptogenic foci in patients with refractory epilepsy remains a significant diagnostic challenge. Magnetic resonance imaging studies frequently fail to reveal an anatomic origin for the seizures, and scalp electroencephalography is often limited to identification of the involved hemisphere. Functional imaging modalities such as PET and SPECT are more promising tools for this application because they reflect the functional pathology associated with the seizure. These changes are more pronounced ictally, but until recently, no radiopharmaceutical was available that could be used routinely for ictal SPECT. The present study was therefore undertaken to determine whether 99mTc-bicisate could be used in ictal SPECT in pediatric patients with refractory epilepsy, to compare the patterns of ictal and interictal blood flow in these patients and to compare the localization information provided by ictal SPECT with that available from other techniques.Technetium-99m-bicisate/SPECT was compared prospectively with scalp EEG for its ability to identify a possible seizure focus in pediatric patients with refractory epilepsy. Ictal and interictal SPECT studies were performed in 10 patients (3-19 yr old, mean age 10.9 +/- 4.3 yr; 7 female, 3 male) in whom MRI scans revealed no lesions that might be responsible for the seizures.Ictal SPECT was performed in all patients, and all ictal studies revealed focal perfusion abnormalities. By comparison, four of the interictal SPECT studies showed regional hypoperfusion that corresponded to the regions of hyperperfusion in the ictal studies, and three showed regional hyperperfusion corresponding to the hyperperfused regions in the ictal studies. Three interictal studies revealed no abnormal perfusion. Scalp EEG provided localization information in five patients.These initial results suggest that ictal SPECT with 99mTc-bicisate is a more promising tool for the identification of epileptogenic foci than interictal SPECT or scalp EEG in patients without focal abnormalities on MRI.
View details for Web of Science ID A1996UV69300015
View details for PubMedID 8965177
Maternal phenylketonuria: Magnetic resonance imaging of the brain in offspring JOURNAL OF PEDIATRICS 1996; 128 (6): 770-775
Phenylketonuria (PKU) produces white matter changes identifiable by magnetic resonance imaging. These changes occur postnatally. Offspring of untreated mothers with PKU also have a brain effect, expressed as microcephaly and mental retardation. This effect occurs prenatally. To determine whether the white matter changes seen in PKU are also present in maternal PKU offspring, despite the different developmental stages of exposure to PKU, we performed brain magnetic resonance imaging studies in seven maternal PKU offspring, five from essentially untreated pregnancies and two from treated pregnancies. None had white matter changes, although the one offspring with PKU had delayed myelination. However, hypoplasia of the corpus callosum was present in three of the four offspring from untreated pregnancies and in the offspring from a maternal PKU pregnancy not treated until the third trimester. Unlike PKU, white matter changes are not a feature of the brain effect in maternal PKU. However, hypoplasia of the corpus callosum is a feature of maternal PKU and is probably a result of inhibition of corpus callosum development at 8 to 20 weeks of gestation. The hypoplastic corpus callosum could be a marker for brain effect in maternal PKU and may have implications for the cognitive deficits in these offspring.
View details for Web of Science ID A1996UQ31900010
View details for PubMedID 8648535
Registration and alignment of three-dimensional images: An interactive visual approach RADIOLOGY 1996; 199 (2): 573-578
A three-dimensional registration and alignment algorithm was developed to align single photon emission computed tomography examinations and magnetic resonance images. Operators manipulated images interactively with real-time visual feedback, with use of both internal and surface features to achieve accurate alignment. Operators aligned five brain phantom examinations (accuracy, 1.6 pixels; consistency, 0.7 pixels) and eight patient brain examinations (consistency, 0.5 pixels).
View details for Web of Science ID A1996UG01100049
View details for PubMedID 8668816
Management of a pediatric hypothalamic mass NEUROSURGERY 1996; 38 (4): 806-811
Structural abnormalities in brain magnetic resonance images of depressed children JOURNAL OF THE AMERICAN ACADEMY OF CHILD AND ADOLESCENT PSYCHIATRY 1996; 35 (3): 307-311
Brain magnetic resonance images (MRIs) of 65 children and adolescents who were hospitalized with depressive disorders (DD) were compared with the brain MRIs of 18 hospitalized psychiatric controls (PC) without a depressive disorder.Volumetric analyses were used to measure frontal lobe volumes (FLV), lateral ventricular volumes (VV), and total cerebral volumes (CV) for all subjects. To correct for differences in absolute cerebral volume associated with different body and head size, the ratios of FLV/CV and VV/CV were used to compare differences between the two groups. A multivariate analysis was used to control for the effects of several independent variables (age, sex, diagnosis).Significant differences were seen in the FLV/CV ratio and the VV/CV ratio when the results were compared between the two groups (DD versus PC). The DD group had a significantly smaller FLV/CV ratio (t = 2.148, df = 79, p = .035) and a significantly larger VV/CV ratio (t = -2.093, df = 79, p = .040).The findings are consistent with previous reports in depressed adults and may implicate the frontal lobes in the pathogenesis of early-onset depressive disorders.
View details for Web of Science ID A1996TW74300011
View details for PubMedID 8714318
Radiologic-pathologic conference of Children's Hospital Boston: Scalp mass in a child following treatment for craniopharyngioma PEDIATRIC RADIOLOGY 1996; 26 (3): 236-238
A five-and-a-half-year-old boy with a history of craniopharyngioma presented with an enlarging scalp mass. The clinical history, CT images, histological findings and relevant discussion are presented.
View details for Web of Science ID A1996UB04600017
View details for PubMedID 8599018
Neuromyelitis optica in childhood: Case report with sequential MRI findings JOURNAL OF CHILD NEUROLOGY 1996; 11 (2): 164-167
Pediatric orbital and visual pathway lesions NEUROIMAGING CLINICS OF NORTH AMERICA 1996; 6 (1): 179-?
This article presents the clinical and imaging aspects of some common and important orbital and optic pathway lesions occurring in childhood. Lesions that arise outside of and encroach on the orbit and visual pathways are included. Abnormalities intrinsic to the globe are covered elsewhere in this issue.
View details for Web of Science ID A1996UN74100011
View details for PubMedID 8919140
Craniopharyngioma: Preliminary results of stereotactic radiation therapy RADIOSURGERY 1995 1996; 1: 75-82
Technetium-99m-HMPAO SPECT in Sturge-Weber syndrome JOURNAL OF NUCLEAR MEDICINE 1996; 37 (1): 81-83
Technetium-99m-HMPAO interictal SPECT was performed on three pediatric patients with Sturge-Weber syndrome (SWS).Brain SPECT of all three patients demonstrated markedly diminished tracer localization in the affected hemisphere. In one patient, the SPECT abnormality was more extensive than the associated abnormalities on CT and MRI.Technetium-99m-HMPAO brain SPECT can detect cerebral perfusion abnormalities associated with SWS and deserves consideration in the imaging evaluation of SWS patients.
View details for Web of Science ID A1996TP05200023
View details for PubMedID 8544008
CYCLOSPORINE NEUROTOXICITY AND ITS RELATIONSHIP TO HYPERTENSIVE ENCEPHALOPATHY - CT AND MR FINDINGS IN 16 CASES AMERICAN JOURNAL OF ROENTGENOLOGY 1995; 165 (3): 627-631
The neurotoxic effects of cyclosporine therapy are well known but poorly understood. Imaging studies typically show subcortical edema predominantly affecting the posterior regions of the brain. We sought to determine the causes for these findings by comparing radiographic data with various clinical parameters.In a 3-year period, 16 patients with neurologic findings attributed to cyclosporine therapy were examined with CT, MR imaging, or both. In most cases, imaging was performed both at the onset of the neurologic syndrome and after it had resolved. The radiographic findings were evaluated with respect to lesion location and changes over time. Various clinical and laboratory data obtained throughout the patients' hospital course were also reviewed, including cyclosporine levels, blood pressure values, hematologic data, and serum levels of cholesterol, magnesium, creatinine, and albumin.The only major factor associated with the neurotoxic effects of cyclosporine in all patients was systemic hypertension. Microangiopathic hemolytic anemia, thrombocytopenia, and hypoalbuminemia were also common, and patients usually displayed signs of sympathetic overactivation. The onset of neurologic symptoms was unrelated to serum levels of creatinine, magnesium, cholesterol, or cyclosporine. The clinical and radiographic findings of these patients were identical to those previously reported in patients with hypertensive encephalopathy. Findings resolved in all but one patient after reduction of blood pressure, with or without reduction in cyclosporine dose. In four patients, intracranial hemorrhages occurred during the hypertensive episode, resulting in one fatality.The clinical and radiologic findings in patients showing the neurotoxic effects of cyclosporine appear to be identical to those with hypertensive encephalopathy. Other associated factors, such as cyclosporine-induced vasculopathy or hypoalbuminemia may also play a role in the condition, and intracranial hemorrhage may occur owing to associated thrombocytopenia. Symptoms generally resolve after reduction of blood pressure, and follow-up is usually unnecessary in uncomplicated cases.
View details for Web of Science ID A1995RQ00600026
View details for PubMedID 7645483
MAGNETIC-RESONANCE IMAGE-DIRECTED STEREOTAXIC NEUROSURGERY - USE OF IMAGE FUSION WITH COMPUTERIZED-TOMOGRAPHY TO ENHANCE SPATIAL ACCURACY JOURNAL OF NEUROSURGERY 1995; 83 (2): 271-276
Distortions of the magnetic field, such as those caused by susceptibility artifacts and peripheral magnetic field warping, can limit geometric precision in the use of magnetic resonance (MR) imaging in stereotactic procedures. The authors have routinely found systematic error in MR stereotactic coordinates with a median of 4 mm compared to computerized tomography (CT) coordinates. This error may place critical neural structures in jeopardy in sme procedures. A description is given of an image fusion technique that uses a chamfer matching algorithm; the advantages of MR imaging in anatomical definition are combined with the geometric precision of CT, while eliminating most of the anatomical spatial distortion of stereotactic MR imaging. A stereotactic radiosurgical case is presented in which the use of MR localization alone would have led to both irradiation of vital neural structures outside the desired target volume and underdose of the intended target volume. The image fusion approach allows for the use of MR imaging, combined with stereotactic CT, as a reliable localizing technique for stereotactic neurosurgery and radiosurgery.
View details for Web of Science ID A1995RL11000014
View details for PubMedID 7616273
HEMORRHAGIC VASCULOPATHY AFTER TREATMENT OF CENTRAL-NERVOUS-SYSTEM NEOPLASIA IN CHILDHOOD - DIAGNOSIS AND FOLLOW-UP AMERICAN JOURNAL OF NEURORADIOLOGY 1995; 16 (4): 693-699
To review the clinical data, imaging findings, and intermediate outcomes of a series of children with hemorrhagic vasculopathy after treatment for intracranial neoplasia.We retrospectively analyzed the medical records and imaging examinations of 20 pediatric patients (ages 1 to 15 years) with intracranial neoplasia in whom delayed intracranial hemorrhage developed after cranial irradiation or radiation combined with systemic or intrathecal chemotherapy. Patients with intracranial hemorrhage from other identifiable causes were excluded. Histopathologic analysis was available in four patients.Twenty patients with delayed intracranial hemorrhage received cranial irradiation alone (n = 9) or combined radiation and chemotherapy (n = 11) for primary brain tumors (n = 13), leukemia (n = 6), or lymphoma (n = 1). Imaging findings were consistent with hemorrhages of varying ages. The hemorrhages were not associated with tumor recurrence nor second tumors. Except for location of the hemorrhage, no significant relationship was established between outcome and original diagnosis, radiation dose (range, 1800 to 6000 centigray), chemotherapeutic agent or dosage, age at treatment, or interval between therapy and hemorrhage (mean, 8.1 years). Only brain stem hemorrhage was associated with a poor outcome.In children with central nervous system neoplasia who have undergone cranial irradiation, or radiation combined with chemotherapy, delayed intracranial hemorrhage may develop.
View details for Web of Science ID A1995QT33400012
View details for PubMedID 7611024
DEVELOPMENTAL AND NEUROLOGIC STATUS OF CHILDREN AFTER HEART-SURGERY WITH HYPOTHERMIC CIRCULATORY ARREST OR LOW-FLOW CARDIOPULMONARY BYPASS NEW ENGLAND JOURNAL OF MEDICINE 1995; 332 (9): 549-555
Deep hypothermia with either total circulatory arrest or low-flow cardiopulmonary bypass is used to support vital organs during heart surgery in infants. We compared the developmental and neurologic sequelae of these two strategies one year after surgery.Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a method of support consisting predominantly of circulatory arrest or a method consisting predominantly of low-flow bypass. Developmental and neurologic evaluations and magnetic resonance imaging (MRI) were performed at one year of age.Of the 171 patients enrolled in the study, 155 were evaluated. After adjustment for the presence or absence of a ventricular septal defect, the infants assigned to circulatory arrest, as compared with those assigned to low-flow bypass, had a lower mean score on the Psychomotor Development Index of the Bayley Scales of Infant Development (a 6.5-point deficit, P = 0.01) and a higher proportion had scores < or = 80 (i.e., 2 SD or more below the population mean) (27 percent vs. 12 percent, P = 0.02). The score on the Psychomotor Development Index was inversely related to the duration of circulatory arrest (P = 0.02). The risk of neurologic abnormalities increased with the duration of circulatory arrest (P = 0.04). The method of support was not associated with the prevalence of abnormalities on MRI scans of the brain, scores on the Mental Development Index of the Bayley Scale, or scores on a test of visual-recognition memory. Perioperative electroencephalographic seizure activity was associated with lower scores on the Psychomotor Development Index (P = 0.002) and an increased likelihood of abnormalities on MRI scans of the brain (P < 0.001).Heart surgery performed with circulatory arrest as the predominant support strategy is associated with a higher risk of delayed motor development and neurologic abnormalities at the age of one year than is surgery with low-flow bypass as the predominant support strategy.
View details for Web of Science ID A1995QJ09000001
View details for PubMedID 7838188
DISSECTION OF THE CAROTID AND VERTEBRAL ARTERIES - IMAGING WITH MR-ANGIOGRAPHY AMERICAN JOURNAL OF ROENTGENOLOGY 1995; 164 (3): 673-677
Arterial dissection occurs when an intimal tear allows blood to enter the arterial wall, potentially compromising the lumen and reducing blood flow. Carotid and vertebral artery dissections typically occur after major trauma, although they also can arise spontaneously or after trivial injury. Arterial dissection has been associated with a variety of factors, including hypertension, fibromuscular dysplasia, Marfan syndrome, cystic medial necrosis, oral contraceptives, drug abuse (sympathomimetics), and infection [1-8]. It is important to recognize arterial dissection early so that prompt treatment can be initiated to prevent ischemic complications . In this essay, we illustrate the use of MR angiography in the diagnosis of carotid and vertebral artery dissection.
View details for Web of Science ID A1995QH86000025
View details for PubMedID 7863892
DYSRAPHIC MYELODYSPLASIAS ASSOCIATED WITH UROGENITAL AND ANORECTAL ANOMALIES - PREVALENCE AND TYPES SEEN WITH MR-IMAGING AMERICAN JOURNAL OF ROENTGENOLOGY 1994; 163 (5): 1199-1203
The purpose of this study was to determine by MR imaging the prevalence and types of dysraphic abnormalities of the spinal cord (i.e., myelodysplasias) associated with urogenital and anorectal malformations of childhood.Since 1987, 92 patients with imperforate anus complex, cloacal malformation, and cloacal exstrophy have had MR imaging as a screening examination for occult dysraphic myelodysplasia. The prevalence and types of myelodysplasia were determined for each group.The prevalence of dysraphic myelodysplasia in each group of children was 17% (1/6) for low imperforate anus (ectopic anus), 34% (11/32) for high imperforate anus (with fistulization), 46% (19/41) for cloacal malformation, and 100% (13/13) for cloacal exstrophy. The most common abnormalities in each group were tethered cord with intradural or filar lipoma in imperforate anus; low-placed or dysplastic conus medullaris and tethered cord with lipoma or myelolipoma in cloacal malformation; and lipomyelocele, lipomyelomeningocele, or lipomyelocystocele in cloacal exstrophy.Our results show that the prevalence of myelodysplasia as seen on MR imaging is high in patients with urogenital and anorectal anomalies.
View details for Web of Science ID A1994PQ81700033
View details for PubMedID 7976901
STEREOTAXIC RADIOTHERAPY FOR PEDIATRIC AND ADULT BRAIN-TUMORS - PRELIMINARY-REPORT INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 1994; 30 (3): 531-539
Stereotactic radiotherapy is a new modality that combines the accurate focal dose delivery of stereotactic radiosurgery with the biological advantages of conventional radiotherapy (1.8-2.0 Gy/day using 25-30 fractions). The modality requires sophisticated treatment planning, dedicated high-energy linear accelerator, and relocatable immobilization devices. We report here our early experience using stereotactic radiotherapy for intracranial neoplasms.Between June 1992 and September 1993, we treated 82 patients with central nervous system lesions using stereotactic radiotherapy, delivered from a dedicated 6 MV stereotactic linear accelerator. A head fixation frame provided daily relocatable setup using a dental plate for all patients over 8 years of age. A modified head frame, which does not require a mouthpiece, was used for children requiring anesthesia. The patients ranged in age from 9 months to 76 years. Thirty-three patients were children less than 21 years of age. Selection criteria for the protocol included: (a) focal, small (< 5 cm) radiographically distinct lesions known to be radiocurable (pituitary adenoma, craniopharyngioma, meningioma, acoustic neuroma, pilocytic astrocytoma, retinoblastoma), and (b) lesions located in regions not amenable to surgery or radiosurgery such as the brain stem or chiasm. Standard fractionation and conventional doses were delivered. Patients with low-grade astrocytoma, oligodendroglioma, or ependymoma were treated using a dose escalation regime consisting of conventional doses plus a 10% increase.Although follow-up is 16 months (range 3-16 months), posttreatment radiographic studies in 77 patients have been consistent with changes similar to those found after conventional radiation therapy. To date, reduction of up to 50% of the original volume has been noted in 19 out of 77 patients, and 4 patients had a complete response, 2 with dysgerminoma, and 1 each with astrocytoma and retinoblastoma. In 56 patients disease was either stable or the follow-up was too short for evaluation. While the follow-up is relatively short, there have been no in-field or marginal recurrences. The only unexpected radiographic findings were in three patients with pilocytic astrocytomas, who developed asymptomatic edema in the treatment volume. Accuracy in daily fractionation was excellent. In over 2000 patient setups with 41,000 scalp measurements, reproducibility was found to be within 0.41 mm (median) of baseline readings, allowing for precise immobilization throughout the treatment course. The treatment in all cases was well tolerated with minimal acute effects. Our stereotactic radiotherapy facility can provide fractionated therapy for 10-12 patients a day efficiently and accurately.The treatment and relocatable stereotactic head frames were well tolerated with minimal acute effects. No long-term sequelae have been noted, although the observation period is short. To fully define the role of stereotactic radiotherapy, we are conducting prospective studies to evaluate neurocognitive and neuroendocrine effects. We expect that this innovative approach will make a significant impact on the treatment of intracranial neoplasms, particularly in children.
View details for Web of Science ID A1994PK60400002
View details for PubMedID 7928483
SURGERY, RADIATION, AND COMBINATION THERAPY IN THE TREATMENT OF CHILDHOOD CRANIOPHARYNGIOMA A 20-YEAR EXPERIENCE PEDIATRIC NEUROSURGERY 1994; 21: 75-81
The records of 61 children treated for primary craniopharyngioma at the Children's Hospital in Boston and The Joint Center for Radiation Therapy from 1970 to 1990 were reviewed to determine if any information could be obtained regarding the efficacy of surgery versus radiotherapy or a combination of these modalities for this tumor. A select group of 9 children treated without biopsy by radiation therapy alone were all alive without disease progression at follow-up. Although 9 of the 15 children who underwent surgery alone had tumor recurrence, 7 of the 37 children treated with radiotherapy following surgery have also recurred. Treatment morbidity occurred in all groups, but the 10-year actuarial survival for all patients was 91%. Early postoperative imaging and pre- and posttreatment endocrine and neuropsychologic evaluations need to be carried out in all prospective studies of the treatment of craniopharyngioma in children.
View details for Web of Science ID A1994PR51800014
View details for PubMedID 7841082
ADVANCES IN RADIATION-THERAPY FOR CRANIOPHARYNGIOMAS PEDIATRIC NEUROSURGERY 1994; 21: 101-107
The overall survival for patients with craniopharyngioma is excellent. However, conventional treatments that include aggressive surgery and standard irradiation have been associated with significant morbidity. Focal radiation treatment with stereotactic radiosurgery has a role in selected cases, but may also be damaging to sensitive normal tissues such as the optic chiasm. Stereotactic radiotherapy (SRT) is a technique that allows for conventionally fractionated radiation under stereotactic guidance. Thus, highly focal and precise radiotherapy is now coupled with fractionation, enabling the treatment of selected tumors with a potentially improved therapeutic index. Dose optimization with SRT for focally discrete tumors should result in equivalent local control and survival compared to patients treated with conventional irradiation. We anticipate a significant decrease in late effects, especially neuropsychological and neuroendocrine sequelae.
View details for Web of Science ID A1994PR51800018
View details for PubMedID 7841067
MICROVESSEL COUNT AND CEREBROSPINAL-FLUID BASIC FIBROBLAST GROWTH-FACTOR IN CHILDREN WITH BRAIN-TUMORS LANCET 1994; 344 (8915): 82-86
Tumour growth is angiogenesis-dependent; brain tumours have more intense neovascularisation than other tumours and produce basic fibroblast growth factor, a potent angiogenic mediator. Because little is known about the release of basic fibroblast growth factor from brain tumours into extracellular fluids, we tested cerebrospinal fluid (CSF) from 26 children and young adults with brain tumours and 18 controls for basic fibroblast growth factor and for proliferative activity on cultured capillary endothelial cells. We also measured the density of microvessels in tumours by immunohistochemical staining. Basic fibroblast growth factor was detected in the CSF of 62% (16 of 26) patients with brain tumours but in none of the controls. Specimens with basic fibroblast growth factor stimulated DNA synthesis of capillary endothelial cells in vitro. Endothelial proliferative activity was blocked by neutralising antibodies to basic fibroblast growth factor. Basic fibroblast growth factor correlated with mitogenic activity in CSF in vitro (p < or = 0.0001), and with density of microvessels in histological sections (p < or = 0.005). A microvessel count of > or = 68 per 200 x field was associated with tumour recurrence (p = 0.005) and with mortality (p = 0.02). Basic fibroblast growth factor in brain tumours may mediate angiogenesis as measured by microvessel density in histological sections, so has potential as both a marker for neoplasia and a target for tumour treatments. Furthermore, evaluation of cerebrospinal fluid basic fibroblast growth factor, along with microvessel quantitation in biopsied tumours, may provide improved prognostic information for the management of patients with brain tumours.
View details for Web of Science ID A1994NV73200008
View details for PubMedID 7516992
Imaging of pediatric central nervous system infections. Neuroimaging clinics of North America 1994; 4 (2): 367-391
IMAGE FUSION FOR STEREOTAXIC RADIOTHERAPY AND RADIOSURGERY TREATMENT PLANNING INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 1994; 28 (5): 1229-1234
We describe an image fusion application that addresses two basic problems that previously limited the use of magnetic resonance imaging (MRI) for geometric localization in stereotactic radiosurgery (SRS) and stereotactic radiotherapy (SRT). The first limitation is imposed by the use of a relocatable, MRI-incompatible, stereotactic frame for stereotactic radiotherapy. The second limitation is an inherent lack of geometric fidelity in current MRI scanners that invalidates the use of MRI for stereotactic localization.We recently developed and implemented a novel automated method for fusing computerized tomography (CT) and MRI volumetric image studies. The method is based on a chamfer matching algorithm, and provides a quality assurance procedure to verify the accuracy of the fused image set. The image fusion protocol removes the need for stereotactic fixation of the patient for the MRI study.The image fusion protocol significantly improves on the spatial accuracy of the MRI study. We demonstrate the effect of distortion and the effectiveness of the fusion with a phantom study. We present two case studies, an acoustic neurinoma treated with SRS, and a pilocytic astrocytoma treated with SRT.The image fusion protocol significantly improves our logistical management of treating patients with radiosurgery and makes conformal therapy practical for treating patients with SRT. The image fusion protocol demonstrates both the superior diagnostic quality and the poor geometric fidelity of MRI. MRI is a required imaging modality in stereotactic therapy. Image fusion combines the superior MRI diagnostic quality with the superior CT geometric definition, and makes the use of MRI in stereotactic therapy possible and practical.
View details for Web of Science ID A1994NG12500023
View details for PubMedID 8175410
STURGE-WEBER SYNDROME WITH POSTERIOR-FOSSA INVOLVEMENT AMERICAN JOURNAL OF NEURORADIOLOGY 1994; 15 (2): 389-392
Investigating the pediatric central nervous system. Current opinion in pediatrics 1993; 5 (6): 643-652
Imaging investigation of the pediatric central nervous system may be considered in terms of structural imaging and functional imaging. Established structural imaging modalities include ultrasonography, radiography, computed tomography, magnetic resonance imaging (MRI), cerebrospinal fluid imaging (eg, myelography), and angiography. Functional imaging technologies include single photon emission computed tomography (SPECT), positron emission tomography, magnetic resonance spectroscopy, and magnetic resonance perfusion imaging. Furthermore, more recent advances in computer software now allow the superimposed display of separate imaging modalities using image fusion techniques. This technique allows the superimposition of structural imaging data with functional imaging data, eg, SPECT and MRI, or the fusion of structural imaging modalities, eg, computed tomography and MRI. In this respect, current practical and investigative applications of structural and functional imaging of the pediatric central nervous system are reviewed.
View details for PubMedID 8124415
PEDIATRIC AND ADOLESCENT OLIGODENDROGLIOMAS AMERICAN JOURNAL OF NEURORADIOLOGY 1993; 14 (6): 1293-1300
To review the clinical and imaging findings in pediatric and adolescent intracranial pure oligodendrogliomas.The clinical, CT, and MR data in 39 surgically proved pure oligodendrogliomas were retrospectively reviewed.The frontal or temporal lobes were involved in 32 (82%) cases. Seventy percent of the tumors were hypodense on CT, three-fourths were hypointense on T1-weighted images, and all were hyperintense on spin-density and T2-weighted images. Fewer than 40% of the lesions demonstrated calcification, and nearly 60% had well-defined margins. Mass effect was seen in fewer than half of the cases, and edema could be separately identified in only one case. Tumor enhancement was seen in fewer than 25%. In 39 cases after partial (3), subtotal (16), or total (20) resection, follow-up studies demonstrated stability over a mean period of 5 years.The findings in this pediatric series of pure oligodendrogliomas (without mixed cell elements) differ from previous adult series in that calcification, contrast enhancement, and edema are seen less frequently. In addition, very slow or no growth is often characteristic, and these patients have an excellent prognosis with surgical resection.
View details for Web of Science ID A1993MG53700004
View details for PubMedID 8279322
MR IN IDIOPATHIC CENTRAL DIABETES-INSIPIDUS OF CHILDHOOD AMERICAN JOURNAL OF NEURORADIOLOGY 1993; 14 (6): 1407-1410
We present the cases of two children with presumed idiopathic central diabetes insipidus whose follow-up MR studies eventually revealed hypothalamic tumors. Thin-section sagittal T1-weighted MR with gadolinium administration is important in the evaluation of these children, and serial examinations are probably necessary.
View details for Web of Science ID A1993MG53700027
View details for PubMedID 8279339
20-YEAR EXPERIENCE IN CHILDHOOD CRANIOPHARYNGIOMA INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 1993; 27 (2): 189-195
The management of craniopharyngioma is controversial, and surgery alone is frequently advocated. The purpose of this study was to assess the long-term impact of various treatments in childhood craniopharyngioma.Sixty-one children < or = 21 years of age at diagnosis were treated for craniopharyngioma at Children's Hospital and the Joint Center for Radiation Therapy in Boston from 1970 to 1990. The median age was 7.5 years (range 10 months-21 years). There were 33 females and 28 males. The median follow-up was 10 years (range 2-20.5 years). Neuroimaging was available for detailed review in 53. Nine children were treated with radiotherapy alone, 15 were treated with surgery alone, and 37 were treated with both surgery and radiotherapy. All patients in the radiotherapy and surgery plus radiotherapy groups were treated with megavoltage radiation with a median dose of 5464 cGy.All nine of the children treated with radiation therapy alone are alive; none have recurred. Nine of the 15 children treated with surgery alone have recurred (p = 0.007 Fisher exact test). Two are alive with disease, and seven are alive without disease after treatment at relapse with radiation therapy, surgery, or both. Seven of the 37 patients treated with surgery plus radiotherapy have recurred. Three of the seven patients are dead of disease, three patients are alive with disease, and one patient is alive without disease after further treatment. The 10-year actuarial overall survival was 91% for all patients. The 10-year actuarial freedom from progression for the surgery group was 31% compared with 100% for patients treated with radiation therapy only (log rank p = 0.01), and 86% for patients treated with surgery plus radiotherapy at diagnosis (p = 0.001). There were two treatment related deaths, both in the surgery plus radiotherapy group. A higher incidence of visual loss and diabetes insipidus was associated with the use of aggressive surgery. The size of the tumor at presentation correlated with an increased risk of recurrence; 5 of 6 patients with tumors > or = 5 cm experienced recurrences while only 6 of 30 recurred when the tumor was < 5 cm.Overall survival in childhood craniopharyngioma is excellent. However, patients treated with surgery alone have a significantly worse freedom from progression when compared to patients treated with surgery and radiation therapy or radiation therapy alone.
View details for Web of Science ID A1993MC22000001
View details for PubMedID 8407391
PREIRRADATION CHEMOTHERAPY AND HYPERFRACTIONATED RADIATION-THERAPY 66 GY FOR CHILDREN WITH BRAIN-STEM TUMORS - A PHASE-II STUDY OF THE PEDIATRIC-ONCOLOGY-GROUP, PROTOCOL 8833 CANCER 1993; 72 (4): 1404-1413
Fewer than 20% of children with intrinsic brain stem tumors survive longer than 2 years. Although some improvement has been noted in recent trials using higher doses of hyperfractionated radiation therapy (HRT), the feasibility of pre-irradiation chemotherapy has not been explored in these patients with poor prognosis.Between February 1988 and March 1989, 37 patients were entered onto a Phase II Pediatric Oncology Group study for evaluating the feasibility, response, and toxicity of treating children with high-risk brain stem tumors with chemotherapy followed by HRT (66 Gy). Chemotherapy consisted of four cycles of cisplatin (100 mg/m2) plus cyclophosphamide (3 g/m2).Of 32 eligible patients, 65% improved clinically during the first 2-3 cycles of chemotherapy; 75% of those improving were weaned from steroids. On neuroradiology review of scans before and after chemotherapy, 3 patients had partial responses (PR, > 50% shrinkage), 23 had stable disease (SD), and 6 had progressive disease (PD). The median survival was 9 months. The three patients who attained a PR on chemotherapy were among the longest survivors at 38 plus, 44 plus, and 40 months. Toxicities included profound but brief marrow suppression, transient electrolyte-renal dysfunction, and ototoxicity. Brain stem swelling from intravenous fluids caused transient deterioration in two patients. Six patients developed an unusual syndrome of transient marrow suppression after HRT.This study suggests that pre-irradiation chemotherapy can be successfully added to the treatment of patients with brain stem tumors with both clinical and objective responses noted, but that other agents must be identified to overcome the apparent development of drug resistance and to improve survival.
View details for Web of Science ID A1993LR31300040
View details for PubMedID 8339231
COMPUTED-TOMOGRAPHY IMAGING IN CHILDREN WITH HEAD TRAUMA - UTILIZATION AND APPROPRIATENESS FROM A QUALITY IMPROVEMENT PERSPECTIVE INFECTION CONTROL AND HOSPITAL EPIDEMIOLOGY 1993; 14 (8): 491-499
Computed tomography (CT) imaging plays an important role in the acute evaluation and management of children with head trauma. When routine quality improvement (QI) meetings with representatives from the Children's Hospital radiology and emergency departments revealed disagreement regarding the utilization and appropriateness of CT in children presenting with head trauma, an interdepartmental QI team was formed to address this issue. Because formal criteria for obtaining CTs for head trauma were unavailable, internal institutional criteria were developed by consensus after literature review. Contrary to perceptions of some staff members, the majority (95%) of children who received CT met at least one of the established criteria over a one-year study period. There was little relationship between the presence of criteria and abnormal CT results, but decisions whether to admit patients to the hospital or to send them home were influenced by CT results. Follow-up studies suggested that patients who were discharged home with a normal CT or no CT had uniformly good outcomes.
View details for Web of Science ID A1993LR11400013
View details for PubMedID 8376744
RADIOLOGIC DETERMINATION OF THE CAUDAL BORDER OF THE SPINAL FIELD IN CRANIAL SPINAL IRRADIATION INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS 1993; 26 (4): 669-673
The purpose of this paper is to determine the inferior border of the caudal sac which dictates the placement of the lower border of the spinal field in Cranial Spinal Irradiation.We have reviewed the pre-treatment craniospinal Magnetic Resonance Imaging studies of 24 evaluable children with seeding central nervous system tumors who were treated at our institution with Cranial Spinal Irradiation since 1988.The Magnetic Resonance Imaging studies demonstrated significant variation in the terminal location of the caudal sac, ranging from S2 to S4. The most frequent termination was at S2 (12/24). In four patients (4/24), termination was at mid S1 and in eight others (8/24), it was found to be at or below S3. In addition, the presence of spinal metastases may displace the distal limit even further inferiorly.Rather than arbitrarily placing the inferior field edge at S2, we recommend individualizing the required margin for the spinal field which should be determined using sagittal T1-weighted images of the lumbosacral spine. This is particularly important in patients who present with spinal metastases, since tumor may extend the dural sac termination distally.
View details for Web of Science ID A1993LP36100015
View details for PubMedID 8330999
TL-201 VERSUS TECHNETIUM-99M-MIBI SPECT IN EVALUATION OF CHILDHOOD BRAIN-TUMORS - A WITHIN-SUBJECT COMPARISON JOURNAL OF NUCLEAR MEDICINE 1993; 34 (7): 1045-1051
We previously found that 201TI SPECT is a highly specific agent for detection of metabolic activity of childhood brain tumors. To compare the relative diagnostic accuracy of 201TI and a technetium-based tumor-avid agent, we have obtained SPECT in 19 children using 201TI (37-111 MBq) followed immediately by 99mTc-methoxyisobutylisonitrile (MIBI) (370-740 MBq) intravenously. Moderate to intense focal uptake of both tracers characterized true positive cases (n = 6). Lesion boundaries were better defined by MIBI. Uptake of MIBI by choroid plexus occurred despite pretreatment with potassium perchlorate (6 mg/kg) and complicated interpretation of deep/paraventricular lesions. Preliminary assessment indicated sensitivity approximately 67% (TI and MIBI); specificity approximately 91% (TI); approximately 100% (MIBI). Two tumors (medulloblastoma, dysgerminoma) were TI/MIBI nonavid. Semi-quantitative assessment of tracer uptake was made using a ratio of radioactivity in tumor-containing areas compared to uninvolved brain. Ratio values were (mean +/- s.d.) 7.88 +/- 7.70 (TI) and 27.1 +/- 36.41 (MIBI). The spectrum of tumor avidity is similar for TI and MIBI. Clearer identification of boundaries by MIBI may be an advantage in applications, e.g., radiotherapy port planning.
View details for Web of Science ID A1993LL23800009
View details for PubMedID 8315477
CYSTIC RETROCEREBELLAR MALFORMATIONS - UNIFICATION OF THE DANDY-WALKER COMPLEX AND THE BLAKE POUCH CYST PEDIATRIC RADIOLOGY 1993; 23 (4): 258-260
Twenty-six cases of developmental retrocerebellar cyst (RCC) formation are studied with respect to determining the usefulness and anatomic relevance of separate terms currently in use, including Dandy-Walker complex, Dandy-Walker malformation, Dandy-Walker variant, mega-cisterna magna (MCM), and Blake's pouch cyst. An anatomic and embryological continuum between Dandy-Walker complex and Blake's pouch cyst is proposed. A method for the useful assessment of RCC is outlined. The patency or closure of the aqueduct is crucial to the evaluation and management of hydrocephalus associated with RCC formation.
View details for Web of Science ID A1993LP93900004
View details for PubMedID 8414749
FAST SPIN-ECHO IMAGING OF INTRACRANIAL NEOPLASMS JOURNAL OF COMPUTER ASSISTED TOMOGRAPHY 1993; 17 (3): 425-431
Our goal was to compare dual echo fast SE (FSE) T2-weighted MRI of intracranial neoplasms with conventional SE (CSE) images. In phase 1 of the study, CSE and FSE dual echo MR studies of 33 patients with intracranial neoplasms and 26 normal controls were separately interpreted by three neuroradiologists blinded to clinical history to ascertain differences in lesion conspicuity. The CSE and FSE images were read independently, in random order, with at least a 3 week interval between readings. In phase 2 of the study, CSE and FSE sequences were compared side by side by three neuroradiologists independently to evaluate lesion conspicuity and artifacts and to determine whether FSE would be an acceptable replacement for CSE imaging. Lesion detection was equivalent in 111 of 117 interpretations (94.9%). The CSE and FSE sequences were equivalent in detecting lesion-associated abnormalities (hemorrhage, calcium, mass effect, edema, and hydrocephalus) and in characterizing lesion size, margins, and signal intensity. Nonspecific T2 white matter hyperintensities were detected more often with CSE, while susceptibility artifacts were less conspicuous on FSE. Ventricular catheters, postoperative soft tissue and bony changes, and postradiation therapy changes were detected equally well on both sequences. In phase 2 of the study, lesion conspicuity and presence of artifacts were felt to be equivalent with the two sequences. The FSE sequences can serve as a rapid, feasible alternative to conventional CSE sequences for intracranial tumor detection.
View details for Web of Science ID A1993LC74300017
View details for PubMedID 8491905
PROGNOSTIC FACTORS IN MEDULLOBLASTOMA, INCLUDING DNA PLOIDY JOURNAL OF CLINICAL ONCOLOGY 1993; 11 (4): 616-622
DNA ploidy status, completeness of surgical resection, use of chemotherapy, adequacy of radiation therapy, metastatic stage, sex, and age at diagnosis were evaluated as predictors of relapse in 58 patients with cerebellar medulloblastoma.Flow cytometry (FCM) and/or image analysis (IA) were used to characterize tumor DNA ploidy. Twelve tumors (21%) were found to be aneuploid, 11 (19%) tetraploid, and 35 (60%) diploid.The most significant predictors of relapse in univariate analyses were the adequacy of radiation (> or = 50 Gy) (P = .02), metastatic staging (P = .05), completeness of resection (P = .085), and DNA ploidy status (diploid/tetraploid v aneuploid; P = .11). When the 52 patients who received > or = 50 Gy were included in a multivariate Cox model analysis, those with diploid/tetraploid tumors had fewer recurrences than those with aneuploid tumors (relative risk, 0.33; 95% confidence interval, 0.12 to 0.89; P = .03). Patients with complete resections (P = .07), or with stage M0 disease (P = .06) had fewer recurrences than other patients, but these factors were not independent predictors of outcome. DNA ploidy status was correlated with age; 10 of the 12 aneuploid tumors were found in children ages 3 to 10 years. Age, sex, and the use of chemotherapy were not prognostically significant in these analyses.The adequacy of radiation dose and DNA ploidy were the most important prognostic factors in this series. Contrary to previous reports, when corrected for adequacy of treatment, DNA aneuploidy was associated with a poor outcome. By multivariate analyses, DNA ploidy was an independent variable, even when controlling for extent of surgical resection and metastatic stage.
View details for Web of Science ID A1993KV51500005
View details for PubMedID 8478656
EPIDURAL HEMATOMAS IN CHILDREN ANNALS OF EMERGENCY MEDICINE 1993; 22 (3): 535-541
To describe the presentation, management, and outcome of children with traumatic epidural hematoma.Retrospective chart review.Fifty-three children diagnosed with traumatic epidural hematoma on computed tomography scan who were treated at Children's Hospital in Boston between 1980 and 1990.Twenty-four of 53 children developed an epidural hematoma after a fall of less than 5 ft. At the time of diagnosis, 51 of 53 children had one or more symptoms of vomiting, headache, or lethargy. Twenty-six patients were alert, 21 were responsive to verbal or painful stimuli, and five were unresponsive or posturing. Twenty-one (40%) had acute neurologic deterioration before surgery; however, 20 (38%) were alert with normal vital signs and neurologic examinations at diagnosis. All patients survived, and at the time of discharge 45 had normal examinations and eight had neurologic abnormalities; at follow-up only four of these eight had persistent (although mild) abnormalities.Although often dramatic in presentation, epidural hematoma may occur after relatively minor head trauma and in alert children with nonfocal neurologic examinations. In our study, incidence of neurologic sequelae increased if abnormal neurologic examination or depressed mental status was present at diagnosis. The outcome of children in this study is improved from that of previous studies, perhaps due to increased use of computed tomography and higher incidence of low- or moderate-impact trauma in this series.
View details for Web of Science ID A1993KQ32800005
View details for PubMedID 8442541
TETHERED SPINAL-CORD IN PATIENTS WITH ANORECTAL AND UROGENITAL MALFORMATIONS PEDIATRIC NEUROSURGERY 1993; 19 (1): 25-30
We reviewed 26 patients with anorectal or urogenital malformations managed by the neurosurgical service at The Children's Hospital for tethered spinal cord as diagnosed by spinal MRI. There were 13 patients with cloacal exstrophy, 5 with the Vater association, 3 with imperforate anus, and 5 with other complex anomalies. Tethering spinal cord lesions included myelocystocele, lipomyelomeningocele, and filum lipoma. We review the clinical course, management, correlation of MRI and intraoperative findings, and outcome of the patients, as well as the embryology which underlies the association of these malformations.
View details for Web of Science ID A1993KD59100005
View details for PubMedID 8422325
ATYPICAL IDIOPATHIC SCOLIOSIS - MR IMAGING EVALUATION RADIOLOGY 1993; 186 (1): 247-253
The authors analyzed the clinical and imaging findings in 30 consecutive pediatric, adolescent, and young adult patients who underwent MR imaging because of atypical features of idiopathic scoliosis. Atypical clinical and plain radiographic features included early onset (n = 4), rapid progression (n = 19), pain (n = 17), other neurologic symptoms or signs (n = 12), associated syndromes (n = 4), a convex left thoracic or thoracolumbar curve (n = 18), a kyphotic component (n = 7), and pedicle thinning (n = 4). MR imaging demonstrated 17 abnormalities in 11 patients: lumbar disk protrusions (n = 1), patulous intradural space (dural ectasia) (n = 3), hydrosyringomyelia (n = 7), Chiari I malformation (n = 5), and cord astrocytoma (n = 1). Significant associations with abnormal MR imaging findings were shown for patients with pain, weakness, abnormal neurologic findings, and atypical curvatures. Furthermore, there was a striking association of convex left thoracic or thoracolumbar scoliosis with hydrosyringomyelia (six of seven cases). On the basis of these results, "atypical" spinal curvatures at radiography and "atypical" clinical features should prompt performance of additional diagnostic studies.
View details for Web of Science ID A1993KD15300050
View details for PubMedID 8416573
PRIMARY ENDODERMAL SINUS TUMOR OF THE ORBIT - MR FINDINGS AMERICAN JOURNAL OF ROENTGENOLOGY 1992; 159 (2): 399-401
RAPID MR IMAGING OF THE PEDIATRIC BRAIN USING THE FAST SPIN-ECHO TECHNIQUE AMERICAN JOURNAL OF NEURORADIOLOGY 1992; 13 (4): 1169-1177
To evaluate diagnostic reliability and to establish optimal scanning techniques of a recently developed Fast Spin-echo MR pulse sequence that allows rapid proton density-weighted and T2-weighted imaging.We compared lesion conspicuity and signal intensity measurements on Fast Spin-echo and conventional spin-echo sequences in 81 patients ranging from 1 week to 25 years in age on a 1.5-T MR unit. A total of 28 Fast Spin-echo dual-echo images (14 slice locations) were obtained in 2:08 minutes with a 256 x 128 matrix or in 3:12 minutes with a 256 x 192 matrix at a TR of 2000 msec and two excitations.Lesion conspicuity and characterization on Fast Spin-echo images compared favorably with conventional spin-echo images in our series when pseudo-TEs of 15 and 90 msec were employed for proton density-weighted and T2-weighted images, respectively. Fast Spin-echo images yielded diagnostic information in four nonsedated patients whose conventional spin-echo images were either degraded by motion or unobtainable. Fat signal remained bright on T2-weighted Fast Spin-echo images. Magnetic-susceptibility effects were slightly reduced with Fast Spin-echo but did not pose any diagnostic problem in our series.Diagnostically reliable rapid dual-echo brain images can be obtained with Fast Spin-echo sequences.
View details for Web of Science ID A1992JD50200028
View details for PubMedID 1636531
FAST SPIN-ECHO MR IMAGING OF THE BRAIN AND SPINE - CURRENT CONCEPTS AMERICAN JOURNAL OF ROENTGENOLOGY 1992; 158 (6): 1313-1320
CAVERNOUS ANGIOMAS OF THE CENTRAL-NERVOUS-SYSTEM IN CHILDREN JOURNAL OF NEUROSURGERY 1992; 76 (1): 38-46
A surgical series of 19 patients under the age of 18 years with pathologically verified cavernous angioma is presented. Most lesions were located in the cerebral hemispheres, but four were in the pons or midbrain, two in the diencephalon, and one in the spinal cord. Fourteen patients presented with an acute or progressing neurological deficit, three with seizures, one infant with irritability, and one with headache alone. Five patients had family histories of vascular malformations of the central nervous system, and five had multiple lesions. Surgery for small or deep lesions was aided considerably by intraoperative ultrasonographic or stereotactic localization techniques. Pathological examination of the resected malformations revealed a complex histology containing not only typical closely approximated cavernous vessels, but also areas of marked proliferation of granulation tissue and partially re-endothelialized hemorrhage, suggesting a mechanism for the apparent growth of certain cavernous angiomas. The postoperative results were good, with only one patient suffering a permanent worsening of neurological status after surgery. Incomplete resection was initially carried out in five patients, two of whom rebled within 1 year after operation. Long-term follow-up findings in these patients have emphasized the unusual history of certain of these malformations.
View details for Web of Science ID A1992GW10200005
View details for PubMedID 1727167
BRAIN HEMORRHAGE - EVALUATION WITH FAST SPIN-ECHO AND CONVENTIONAL DUAL SPIN-ECHO IMAGES RADIOLOGY 1992; 182 (1): 53-58
Signal intensity of blood products on proton-density- and T2-weighted images obtained with spin-echo (SE) and fast SE (FSE) sequences was evaluated in 15 patients with central nervous system hemorrhage to determine the extent of differences between the two techniques when signal loss from magnetic susceptibility effects in hemorrhagic lesions is considered. Within operator-defined regions of interest, signal intensity of hemorrhage, iron-containing nuclei, white matter, scalp fat, and noise was measured along the phase-encoding direction. Hemosiderin, deoxyhemoglobin, and iron-containing nuclei had slightly higher signal intensity on FSE images than on SE images, but the differences were not statistically significant. Signal intensity of methemoglobin was similar with both sequences, whereas that of scalp fat was higher on FSE images. Signal intensity measurements for most tissues studied were comparable, but the signal-to-noise ratios with FSE imaging were less than those with SE imaging. Although paramagnetic blood products may show slightly higher signal intensity with FSE imaging, contrast with the two sequences was comparable and lesion conspicuity was nearly identical.
View details for Web of Science ID A1992GW05400013
View details for PubMedID 1727309
MR DIAGNOSIS OF BRAIN-DEATH AMERICAN JOURNAL OF NEURORADIOLOGY 1992; 13 (1): 65-66
BIOLOGICAL CLASSIFICATION OF SOFT-TISSUE VASCULAR ANOMALIES - MR CORRELATION AMERICAN JOURNAL OF ROENTGENOLOGY 1991; 157 (3): 559-564
TL-201/TC99M-HMPAO SPECT IMAGING OF TREATED CHILDHOOD BRAIN-TUMORS PEDIATRIC NEUROLOGY 1991; 7 (4): 249-257
To assess whether thallium-201 thallous chloride (Tl) can detect childhood tumors and whether diagnostic effectiveness improves with combined blood flow imaging, 28 children (1.0-18.6 years) were studied using single photon emission computed tomography (SPECT): Tl (1.3-1.8 mCi intravenously), followed in 13 of the patients by technetium-99m-hexamethylpropyleneamine oxime (99mTc-HMPAO; 8-18 mCi intravenously). Tl-uptake was markedly increased with histologically confirmed recurrent brain tumors (N = 12). Tl-avid tumors comprised several histologic types, including 6 astrocytomas/gliomas as well as nonastrocytic neoplasms, such as medulloblastoma and ependymoma. A questionable false-positive study was observed with a treated medulloblastoma. Tl failed to detect 5 tumors (i.e., 2 medulloblastomas, 1 ependymoma, 1 malignant schwannoma, and initially 1 low-grade astrocytoma). The sensitivity and specificity of 201Tl-SPECT for detection of childhood brain tumors was 76.9% and 93.3%, respectively. The mean tumor-to-normal brain ratio for Tl was 2.5 +/- 0.5 (N = 7). In some of the patients, 201Tl-SPECT allowed a more precise assessment of the functional state of the tumor than was possible with computed tomography and magnetic resonance imaging. HMPAO distribution was variously normal, increased or decreased at the site of tissue abnormality, and abnormal blood flow was demonstrated in the remaining neuraxis, in 3 of the 7 patients. Changes in tissue perfusion did not correlate with Tl findings, but were evaluated in only one false-negative study.
View details for Web of Science ID A1991GG17100003
View details for PubMedID 1930415
THE PROGNOSTIC-SIGNIFICANCE OF POSTOPERATIVE RESIDUAL TUMOR IN EPENDYMOMA NEUROSURGERY 1991; 28 (5): 666-672
Between 1970 and 1989, 29 patients with intracranial ependymomas were evaluated and treated at the Children's Hospital in Boston. With a median follow-up of 82 months, the actuarial survival rates at 5 and 10 years were 61 +/- 10% and 46 +/- 12%, respectively. Anaplastic histological findings were uncommon (2 of 29). Initial postoperative radiotherapy was given to 25 patients, with a median tumor dose of 5360 cGy. With a median time to recurrence of 22 months, local failure (within 2 cm of original enhancing mass) was the predominant pattern of relapse (15 of 16 failures). The presence of radiographic residual disease seen on postoperative magnetic resonance imaging or computed tomographic scans was the most important prognostic variable for patients with intracranial ependymoma. Analysis of the 19 patients who underwent postoperative imaging revealed a 75 +/- 15% 5-year freedom from progressive disease for 9 patients with no residual disease, as compared with 0% freedom from progressive disease for the 10 patients with gross residual disease (P = 0.03). In contrast, the surgical assessment of residual disease was not significant (P = 0.4). Age at presentation was also a significant prognostic factor. The overall actuarial survival rate at 12 years for infants 24 months or younger at diagnosis was 0%, as compared with 62 +/- 13% for older patients (P = 0.03). For non-anaplastic ependymomas, complete surgical resection followed by local-field, high-dose (greater than 54 Gy) radiotherapy appears to offer the greatest chance for long-term survival. Because of the markedly reduced survival rate for patients with radiologically apparent postoperative disease, maximal surgical resection and novel therapeutic endeavors appear warranted for this high-risk group. Future protocols should use postoperative imaging, not operative reports, to stratify patients with ependymoma.
View details for Web of Science ID A1991FJ82800005
View details for PubMedID 1876244
CEREBRAL VENOGRAPHY WITH MR RADIOLOGY 1991; 178 (2): 453-458
The authors describe a two-dimensional time-of-flight magnetic resonance (MR) angiography technique to create projection venograms of the head. The technique was applied to 27 healthy volunteers and 39 patients. The superior sagittal and straight sinuses, the internal cerebral veins, and the Galen vein were visualized in all the volunteers. Other veins were seen in a high percentage of subjects. Systematic comparison of digital subtraction angiography (DSA) after intraarterial contrast medium injection and MR venography in patients showed good correlation between the two techniques. MR venography proved helpful in identifying thrombosis or patency of cerebral veins and sinuses and showed collateral venous drainage and venous drainage from arteriovenous malformations. There was good correlation between conventional contrast angiography and MR venography. In conclusion, MR venography is considered reliable for showing the cerebral venous system and provides information additional to that of conventional spin-echo imaging.
View details for Web of Science ID A1991EU03500032
View details for PubMedID 1987608
DEFINITIVE RADIATION-THERAPY IN THE MANAGEMENT OF SYMPTOMATIC PATIENTS WITH OPTIC GLIOMA - SURVIVAL AND LONG-TERM EFFECTS CANCER 1990; 65 (1): 45-52
We reviewed 24 children who had symptomatic gliomas that involved the optic chiasm and were treated with definitive radiation therapy from 1971 to 1986. In eight patients (33%), histologic confirmation of low-grade astrocytoma was obtained. Patients had radiation therapy only if there was evidence of visual deterioration or other clinical or radiographic evidence of disease progression. Radiation doses ranged from 4500 to 5660 cGy (median, 5400 cGy) with up to a 17-year follow-up period (median, 6 years). The 6-year actuarial freedom from disease progression and overall 6-year survival are 88% and 100%, respectively. Visual improvement or stabilization was seen in 21 (91%) patients after radiation. A high incidence of endocrine abnormalities is reported, with 15 of the 18 patients evaluated after treatment showing growth hormone deficiency. We conclude that definitive radiation therapy is associated with tumor control in most of the patients with progressive optic chiasm gliomas. However, neuropsychiatric and endocrine abnormalities are significant problems that need additional evaluation in these children.
View details for Web of Science ID A1990CG42700009
View details for PubMedID 2104571
MR DIAGNOSIS OF DURAL VENOUS SINUS THROMBOSIS COMPLICATING L-ASPARAGINASE THERAPY COMPUTERIZED MEDICAL IMAGING AND GRAPHICS 1989; 13 (4): 319-327
Dural venous sinus thrombosis is an important complication of L-asparaginase chemotherapy. The diagnosis and followup of this condition, using spin echo and fast imaging techniques, is described in three patients. Magnetic Resonance (MR) imaging is a rapid, noninvasive technique for diagnosis and follow-up of this condition. Fast imaging techniques can improve the assessment of these patients.
View details for Web of Science ID A1989AE50100002
View details for PubMedID 2743289
RESULTS OF PRESUMED CRYPTOGENIC EPILEPSY IN CHILDHOOD BY CT SCANNING ANNALES DE RADIOLOGIE 1979; 22 (2-3): 184-187