Publications by authors named "Bruno P Soares"

62 Publications

Wavelet Autoregulation Monitoring Identifies Blood Pressures Associated With Brain Injury in Neonatal Hypoxic-Ischemic Encephalopathy.

Front Neurol 2021 28;12:662839. Epub 2021 Apr 28.

Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, United States.

Dysfunctional cerebrovascular autoregulation may contribute to neurologic injury in neonatal hypoxic-ischemic encephalopathy (HIE). Identifying the optimal mean arterial blood pressure (MAPopt) that best supports autoregulation could help identify hemodynamic goals that support neurologic recovery. In neonates who received therapeutic hypothermia for HIE, we hypothesized that the wavelet hemoglobin volume index (wHVx) would identify MAPopt and that blood pressures closer to MAPopt would be associated with less brain injury on MRI. We also tested a correlation-derived hemoglobin volume index (HVx) and single- and multi-window data processing methodology. Autoregulation was monitored in consecutive 3-h periods using near infrared spectroscopy in an observational study. The neonates had a mean MAP of 54 mmHg (standard deviation: 9) during hypothermia. Greater blood pressure above the MAPopt from single-window wHVx was associated with less injury in the paracentral gyri ( = 0.044; = 63), basal ganglia ( = 0.015), thalamus ( = 0.013), and brainstem ( = 0.041) after adjustments for sex, vasopressor use, seizures, arterial carbon dioxide level, and a perinatal insult score. Blood pressure exceeding MAPopt from the multi-window, correlation HVx was associated with less injury in the brainstem ( = 0.021) but not in other brain regions. We conclude that applying wavelet methodology to short autoregulation monitoring periods may improve the identification of MAPopt values that are associated with brain injury. Having blood pressure above MAPopt with an upper MAP of ~50-60 mmHg may reduce the risk of brain injury during therapeutic hypothermia. Though a cause-and-effect relationship cannot be inferred, the data support the need for randomized studies of autoregulation and brain injury in neonates with HIE.
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http://dx.doi.org/10.3389/fneur.2021.662839DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8113412PMC
April 2021

ACR Appropriateness Criteria® Seizures-Child.

J Am Coll Radiol 2021 May;18(5S):S199-S211

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

In children, seizures represent an extremely heterogeneous group of medical conditions ranging from benign cases, such as a simple febrile seizure, to life-threatening situations, such as status epilepticus. Underlying causes of seizures also represent a wide range of pathologies from idiopathic cases, usually genetic, to a variety of acute and chronic intracranial or systemic abnormalities. This document discusses appropriate utilization of neuroimaging tests in a child with seizures. The clinical scenarios in this document take into consideration different circumstances at the time of a child's presentation including the patient's age, precipitating event (if any), and clinical and electroencephalogram findings and include neonatal seizures, simple and complex febrile seizures, post-traumatic seizures, focal seizures, primary generalized seizures in a neurologically normal child, and generalized seizures in neurologically abnormal child. This practical approach aims to guide clinicians in clinical decision-making and to help identify efficient and appropriate imaging workup. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2021.02.020DOI Listing
May 2021

Neurocutaneous Melanosis: Prenatal Presentation as a Posterior Fossa Cyst.

Neuropediatrics 2021 Jan 14. Epub 2021 Jan 14.

Neuroradiology Division, The University of Vermont Medical Center, Burlington, Vermont, United States.

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http://dx.doi.org/10.1055/s-0040-1722676DOI Listing
January 2021

Pediatric Posterior Fossa Medulloblastoma: The Role of Diffusion Imaging in Identifying Molecular Groups.

J Neuroimaging 2020 07 12;30(4):503-511. Epub 2020 Jun 12.

Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN.

Background And Purpose: The molecular groups WNT activated (WNT), Sonic hedgehog activated (SHH), group 3, and group 4 are biologically and clinically distinct forms of medulloblastoma. We evaluated apparent diffusion coefficient (ADC) values' utility in differentiating/predicting medulloblastoma groups at the initial diagnostic imaging evaluation and prior to surgery.

Methods: We retrospectively measured the ADC values of the enhancing, solid portion of the tumor (EST) and of the whole tumor (WT) and performed Kruskal-Wallis testing to compare the absolute tumor ADC values and cerebellar and thalamic ratios of three medulloblastoma groups (WNT, SHH, and group 3/group 4 combined).

Results: Ninety-three children (65 males) were included. Fifty-seven children had group 3/group 4, 27 had SHH, and 9 had WNT medulloblastomas. The median absolute ADC values in the EST and WT were .719 × 10 and .864 × 10 mm /s for group 3/group 4; .660 × 10 and .965 × 10 mm /s for SHH; and .594 × 10 and .728 × 10 mm /s for WNT medulloblastomas (P = .02 and .13). The median ratio of ADC values in the EST or the WT to normal cerebellar tissue was highest for group 3/group 4 and lowest for WNT medulloblastomas (P = .03 and .09), with similar results in pairwise comparisons of the corresponding thalamic ADC values (P = .02 and .06).

Conclusion: ADC analysis of a tumor's contrast-enhancing solid portion may aid preoperative molecular classification/prediction of pediatric medulloblastomas and may facilitate optimal surgical treatment planning, reducing surgery-induced morbidity.
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http://dx.doi.org/10.1111/jon.12704DOI Listing
July 2020

ACR Appropriateness Criteria® Cerebrovascular Disease-Child.

J Am Coll Radiol 2020 May;17(5S):S36-S54

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Stroke is an uncommon but an important and under-recognized cause of morbidity and mortality in children. Strokes may be due to either brain ischemia or intracranial hemorrhage. Common symptoms of pediatric acute stroke include headache, vomiting, focal weakness, numbness, visual disturbance, seizures, and altered consciousness. Most children presenting with an acute neurologic deficit do not have an acute stroke, but have symptoms due to stroke mimics which include complicated migraine, seizures with postictal paralysis, and Bell palsy. Because of frequency of stroke mimics, in children and the common lack of specificity in symptoms, the diagnosis of a true stroke may be delayed. There are a relatively large number of potential causes of stroke mimic and true stroke. Consequently, imaging plays a critical role in the assessment of children with possible stroke and especially in children who present with acute onset of stroke symptoms. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.01.036DOI Listing
May 2020

ACR Appropriateness Criteria® Head Trauma-Child.

J Am Coll Radiol 2020 May;17(5S):S125-S137

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Head trauma is a frequent indication for cranial imaging in children. The majority of accidental pediatric head trauma is minor and sustained without intracranial injury. Well-validated pediatric-specific clinical decision guidelines should be used to identify very low-risk children who can safely forgo imaging. In those who require acute imaging, CT is considered the first-line imaging modality for suspected intracranial injury because of the short duration of the examination and its high sensitivity for acute hemorrhage. MRI can accurately detect traumatic complications, but often necessitates sedation in children, owing to the examination length and motion sensitivity, which limits rapid assessment. There is a paucity of literature regarding vascular injuries in pediatric blunt head trauma and imaging is typically guided by clinical suspicion. Advanced imaging techniques have the potential to identify changes that are not seen by standard imaging, but data are currently insufficient to support routine clinical use. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2020.01.026DOI Listing
May 2020

Meet in the middle: a technique for resecting nasocranial dermoids-technical note and review of the literature.

Childs Nerv Syst 2020 03 10;36(3):477-484. Epub 2020 Jan 10.

Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Introduction: Nasal dermoids with intracranial extension are benign lesions that can have life-threatening consequences, as a nasal punctum may serve as a portal of entry for infection into the central nervous system. The surgical management of these lesions can be challenging, as the extracranial and intracranial cysts and sinus tract must be resected to prevent recurrence, and the surgery must be carried out with an acceptable esthetic result.

Technique: The authors present a technique for the resection of nasocranial dermoids that eliminates the need to remove and replace the frontonasal segment. Working through a small frontal craniotomy enables the surgeon to drill out the sinus tract through the foramen cecum and inferiorly into the nose. This exposure eliminates the need to enter the frontal sinus, if it is present.

Conclusion: The extracranial punctum and tract are resected from below in a minimally invasive fashion that permits removal of the lesion without a disfiguring scar.
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http://dx.doi.org/10.1007/s00381-020-04499-5DOI Listing
March 2020

Early neurodevelopmental outcome in preterm posthemorrhagic ventricular dilatation and hydrocephalus: Neonatal ICU Network Neurobehavioral Scale and imaging predict 3-6-month motor quotients and Capute Scales.

J Neurosurg Pediatr 2019 Dec 20:1-11. Epub 2019 Dec 20.

7Neurology and Developmental Medicine and.

Objective: Brain injury remains a serious complication of prematurity. Almost half of infants with severe intraventricular hemorrhage (IVH) develop posthemorrhagic ventricular dilatation (PHVD) and 20% need surgery for posthemorrhagic hydrocephalus (PHH). This population is associated with an increased risk of later neurodevelopmental disability, but there is uncertainty about which radiological and examination features predict later disability. In this study the authors sought to devise and describe a novel combination of neurobehavioral examination and imaging for prediction of neurodevelopmental disability among preterm infants with PHVD and PHH.

Methods: The study patients were preterm infants (< 36 weeks gestation) with IVH and PHVD, with or without PHH. Ventricular index (VI), anterior horn width (AHW), thalamooccipital distance (TOD), ventricle/brain (V/B) ratio, and resistive indices (RIs) were recorded on the head ultrasound (HUS) just prior to surgery, or the HUS capturing the worst PHVD when surgery was not indicated. The posterior fossa was assessed with MRI. Neonatal ICU Network Neurobehavioral Scale (NNNS) examinations were performed at term age equivalent for each infant. A neurodevelopmental assessment using the Capute Scales (Capute Cognitive Adaptive Test [CAT] scores and Capute Clinical Linguistic Auditory Milestone Scale [CLAMS] scores) and a motor quotient (MQ) assessment were performed between 3 and 6 months of age corrected for degree of prematurity (corrected age). MQs < 50 reflect moderate to severe delays in early motor milestone attainment, CAT scores < 85 reflect delays in early visual and problem-solving abilities, and CLAMS scores < 85 reflect delays in early language.

Results: Twenty-one infants underwent assessments that included imaging and NNNS examinations, Capute Scales assessments, and MQs. NNNS nonoptimal reflexes (NOR) and hypertonicity subscores and AHW were associated with MQs < 50: NOR subscore OR 2.46 (95% CI 1.15-37.6, p = 0.034), hypertonicity subscore OR 1.68 (95% CI 1.04-3.78, p = 0.037), and AHW OR 1.13 (95% CI 1.01-1.39, p = 0.041). PVHI, cystic changes, and neurosurgical intervention were associated with CAT scores < 85: PVHI OR 9.2 (95% CI 1.2-73.2, p = 0.037); cystic changes OR 12.0 (95% CI 1.0-141.3, p = 0.048), and neurosurgical intervention OR 11.2 (95% CI 1.0-120.4, p = 0.046). Every 1-SD increase in the NOR subscore was associated with an increase in odds of a CAT score < 85, OR 4.0 (95% CI 1.0-15.0, p = 0.044). Worse NNNS NOR subscores were associated with early language delay: for a 1-SD increase in NOR subscore, there was an increase in the odds of a CLAMS score < 85, OR 19.5 (95% CI 1.3-303, p = 0.034).

Conclusions: In former preterm children with severe IVH and PHVD, neonatal neurological examination findings and imaging features are associated with delays at 3-6 months in motor milestones, visual and problem-solving abilities, and language.
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http://dx.doi.org/10.3171/2019.9.PEDS19438DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305038PMC
December 2019

Peering Into Peer Review: Neuroradiology Reviewers Discuss Their Approaches to Assessing a Manuscript.

AJR Am J Roentgenol 2020 01 31;214(1):45-49. Epub 2019 Oct 31.

Department of Radiology, University of Vermont Medical Center, Burlington, VT.

This article provides comments from a small group of highly qualified reviewers of the () regarding their approach to assessing manuscripts. The objective is to educate authors about the issues to which reviewers particularly attend and about errors that will decrease the likelihood of publication. By following the advice provided in this article, authors should be able to compose better manuscripts and reviewers should be able to generate better reviews.
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http://dx.doi.org/10.2214/AJR.19.21999DOI Listing
January 2020

Congenital Oral Masses: An Anatomic Approach to Diagnosis.

Radiographics 2019 Jul-Aug;39(4):1143-1160

From the Department of Radiology and Biomedical Imaging, University of California, San Francisco, 505 Parnassus Avenue, San Francisco, CA (M.C.C., Y.L., D.J.S., O.A.G., C.M.G., J.L.C.) and the Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD (B.P.S.).

Although congenital oral masses are rare, they are readily detectable during fetal US screening. Most congenital oral masses are benign, but some may cause mechanical airway obstruction, resulting in poor outcomes at delivery. The radiologist's ability to describe these abnormalities and their physiologic sequelae accurately can have a substantial effect on perinatal treatment. Furthermore, despite being rare, congenital oral lesions encountered at screening and at follow up fetal MRI provide the opportunity to make a specific diagnosis by following a simple anatomic approach. This article describes an anatomic algorithm as the framework for accurate diagnosis of congenital oral lesions. The imaging appearance of the most common congenital oral cavity neoplasms is outlined, including vascular anomalies, epulides, choristomas, congenital lingual thyroid anomalies, lingual hamartomas, and epignathi, and other conditions that mimic these at US. Also reviewed are perinatal management of masses that affect the fetal airway and the imaging features key to optimizing delivery outcomes. RSNA, 2019.
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http://dx.doi.org/10.1148/rg.2019180128DOI Listing
May 2020

Preschool Language Outcomes following Perinatal Hypoxic-Ischemic Encephalopathy in the Age of Therapeutic Hypothermia.

Dev Neurosci 2019 Jun 5:1-11. Epub 2019 Jun 5.

Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland, USA.

Early studies following perinatal hypoxic-ischemic encephalopathy (HIE) suggested expressive language deficits and academic difficulties, but there is only limited detailed study of language development in this population since the widespread adoption of therapeutic hypothermia (TH). Expressive and receptive language testing was performed as part of a larger battery with 45 children with a mean age of 26 months following perinatal HIE treated with TH. Overall cohort outcomes as well as the effects of gender, estimated household income, initial pH and base excess, and pattern of injury on neonatal brain MRI were assessed. The cohort overall demonstrated expressive language subscore, visual-reception subscore, and early learning composite scores significantly below test norms, with relative sparing of receptive language subscores. Poorer expressive language manifested as decreased vocabulary size and shorter utterances. Expressive language subscores showed a significant gender effect, and estimated socioeconomic status showed a significant effect on both receptive and expressive language subscores. Initial blood gas markers and modified Sarnat scoring did not show a significant effect on language subscores. Binarized MRI abnormality predicted a significant effect on both receptive and expressive language subscores; the presence of specific cortical/subcortical abnormalities predicted receptive language deficits. Overall, the language development profile of children following HIE in the era of hypothermia shows a relative strength in receptive language. Gender and socioeconomic status predominantly predict expressive language deficits; abnormalities detectable on MRI predominantly predict receptive language deficits.
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http://dx.doi.org/10.1159/000499562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6893079PMC
June 2019

ACR Appropriateness Criteria Suspected Spine Trauma-Child.

J Am Coll Radiol 2019 May;16(5S):S286-S299

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Choosing the appropriate imaging in children with accidental traumatic spine injuries can be challenging because the recommendations based on scientific evidence at this time differ from those applied in adults. This differentiation is due in part to differences in anatomy and physiology of the developing spine. This publication uses scientific evidence and a panel of pediatric experts to summarize best current imaging practices for children with accidental spine trauma. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2019.02.003DOI Listing
May 2019

ACR Appropriateness Criteria Scoliosis-Child.

J Am Coll Radiol 2019 May;16(5S):S244-S251

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Scoliosis is frequently encountered in childhood, with prevalence of 2%. The majority is idiopathic, without vertebral segmentation anomaly, dysraphism, neuromuscular abnormality, skeletal dysplasia, tumor, or infection. As a complement to clinical assessment, radiography is the primary imaging modality used to classify scoliosis and subsequently monitor its progression and response to treatment. MRI is utilized selectively to assess for neural axis abnormalities in those at higher risk, including those with congenital scoliosis, early onset idiopathic scoliosis, and adolescent idiopathic scoliosis with certain risk factors. CT, although not routinely employed in the initial evaluation of scoliosis, may have a select role in characterizing the bone anomalies of congenital scoliosis and in perioperative planning. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2019.02.018DOI Listing
May 2019

The Relationship Between Clinical Imaging and Neurobehavioral Assessment in Posthemorrhagic Ventricular Dilation of Prematurity.

Front Physiol 2019 11;10:64. Epub 2019 Feb 11.

Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, MD, United States.

Neonatal intraventricular hemorrhage (IVH) and subsequent posthemorrhagic ventricular dilation and hydrocephalus of prematurity are associated with brain injury and neurodevelopmental impairment in the preterm population. Neuroimaging assesses cerebral injury and guides neurosurgical intervention; however, the relationship of head ultrasound (HUS) and magnetic resonance imaging (MRI) parameters to neonatal exams in this group has not been well described. The NICU Network Neurobehavioral Scale (NNNS) is a reproducible, highly reliable battery with motor and cognitive domain scores. To evaluate the relationship between neonatal neurobehavioral findings on the NNNS and measures of ventricular dilation and associated brain injury on HUS and MRI. Neonates with IVH and ventricular dilatation with and without posthemorrhagic hydrocephalus were enrolled. NNNS exams were performed at approximately term age equivalent. HUS indices were measured on the last HUS before initial neurosurgical procedure or that with worst ventriculomegaly if no intervention. The posterior fossa was assessed with MRI at term. Descriptive statistics including medians, interquartile ranges, means, and percentages were performed. Correlations were estimated using Pearson's method. 28 patients had NNNS and HUS, and 18 patients also had an MRI. Ventricle size measures for the cohort were significantly above normal. Motor and cognitive subscores on the NNNS exam varied from established baseline scores for postmenstrual age. Children who required neurosurgical intervention had higher ventricle/brain ratios and worse NNNS habituation scores. Ventricle sizes were modestly correlated with motor abnormalities (0.24-0.59); larger anterior horn width correlated with nonoptimal reflexes, hypertonicity and hypotonicity. Ventricle sizes were modestly correlated with cognitive scores (-0.44 to 0.27); larger ventricular index correlated with worse attention. Periventricular hemorrhagic infarction correlated with worse habituation. For this cohort of preterm infants with IVH, surgical intervention for posthemorrhagic hydrocephalus correlated with both larger degrees of ventriculomegaly and worse NNNS exams. Findings on both HUS and MRI correlated with motor and cognitive abnormalities on neonatal neurobehavioral exam, suggesting that larger neonatal ventricle sizes and white matter injury have detectable correlates on exam. The NNNS exam provides important additional information when assessing posthemorrhagic ventricular dilation and hydrocephalus of prematurity.
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http://dx.doi.org/10.3389/fphys.2019.00064DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378306PMC
February 2019

The Rare Neurocutaneous Disorders: Update on Clinical, Molecular, and Neuroimaging Features.

Top Magn Reson Imaging 2018 Dec;27(6):433-462

Division of Pediatric Radiology and Pediatric Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD.

Phakomatoses, also known as neurocutaneous disorders, comprise a vast number of entities that predominantly affect structures originated from the ectoderm such as the central nervous system and the skin, but also the mesoderm, particularly the vascular system. Extensive literature exists about the most common phakomatoses, namely neurofibromatosis, tuberous sclerosis, von Hippel-Lindau and Sturge-Weber syndrome. However, recent developments in the understanding of the molecular underpinnings of less common phakomatoses have sparked interest in these disorders. In this article, we review the clinical features, current pathogenesis, and modern neuroimaging findings of melanophakomatoses, vascular phakomatoses, and other rare neurocutaneous syndromes that may also include tissue overgrowth or neoplastic predisposition.
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http://dx.doi.org/10.1097/RMR.0000000000000185DOI Listing
December 2018

ACR Appropriateness Criteria Sinusitis-Child.

J Am Coll Radiol 2018 Nov;15(11S):S403-S412

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Sinusitis is common in children that usually resolves spontaneously. Imaging is not part of the standard of care for initial diagnosis, however may be necessary in cases with persistent or chronic sinusitis to guide surgical intervention, or to rule out intracranial and vascular complications of sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) are the leading imaging modalities. In this article, appropriateness in use of imaging modalities are discussed under common/clinically relevant scenarios. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2018.09.029DOI Listing
November 2018

Thalamic and dentate nucleus abnormalities in the brain of children with Gaucher disease.

Neuroradiology 2018 Dec 17;60(12):1353-1356. Epub 2018 Oct 17.

Department of Neuroradiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Gaucher disease (GD) represents the most common lysosomal storage defect. It is classified into three phenotypes: type 1 non-neuronopathic, type 2 acute neuronopathic, and type 3 subacute/chronic neuronopathic. Although children affected by GD may present with a broad spectrum of neurological signs, brain magnetic resonance imaging (MRI) findings are usually normal or non-specific. We report three cases of GD with previously undescribed brain MRI changes mainly affecting the thalami and/or the dentate nuclei. We discuss the possible etiopathogenesis of these abnormalities. Correlation between brain MRI abnormalities, neurological symptoms, and treatment efficacy is still unclear.
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http://dx.doi.org/10.1007/s00234-018-2116-zDOI Listing
December 2018

ST3GAL5-Related Disorders: A Deficiency in Ganglioside Metabolism and a Genetic Cause of Intellectual Disability and Choreoathetosis.

J Child Neurol 2018 11 5;33(13):825-831. Epub 2018 Sep 5.

1 Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, MD, USA.

GM3 synthase deficiency is due to biallelic pathogenic variants in ST3GAL5, which encodes a sialyltransferase that synthesizes ganglioside GM3. Key features of this rare autosomal recessive condition include profound intellectual disability, failure to thrive and infantile onset epilepsy. We expand the phenotypic spectrum with 3 siblings who were found by whole exome sequencing to have a homozygous pathogenic variant in ST3GAL5, and we compare these cases to those previously described in the literature. The siblings had normal birth history, subsequent developmental stagnation, profound intellectual disability, choreoathetosis, failure to thrive, and visual and hearing impairment. Ichthyosis and self-injurious behavior are newly described in our patients and may influence clinical management. We conclude that GM3 synthase deficiency is a neurodevelopmental disorder with consistent features of profound intellectual disability, choreoathetosis, and deafness. Other phenotypic features have variable expressivity, including failure to thrive, epilepsy, regression, vision impairment, and skin findings. Our analysis demonstrates a broader phenotypic range of this potentially under-recognized disorder.
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http://dx.doi.org/10.1177/0883073818791099DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6188822PMC
November 2018

Preterm neuroimaging and neurodevelopmental outcome: a focus on intraventricular hemorrhage, post-hemorrhagic hydrocephalus, and associated brain injury.

J Perinatol 2018 11 30;38(11):1431-1443. Epub 2018 Aug 30.

Neurosciences Intensive Care Nursery, The Johns Hopkins School of Medicine, Baltimore, MD, USA.

Intraventricular hemorrhage in the setting of prematurity remains the most common cause of acquired hydrocephalus. Neonates with progressive post-hemorrhagic hydrocephalus are at risk for adverse neurodevelopmental outcomes. The goal of this review is to describe the distinct and often overlapping types of brain injury in the preterm neonate, with a focus on neonatal hydrocephalus, and to connect injury on imaging to neurodevelopmental outcome risk. Head ultrasound and magnetic resonance imaging findings are described separately. The current state of the literature is imprecise and we end the review with recommendations for future radiologic and neurodevelopmental research.
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http://dx.doi.org/10.1038/s41372-018-0209-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215507PMC
November 2018

Cerebral Diffusivity Changes in Fetuses with Chiari II Malformation.

Fetal Diagn Ther 2019 17;45(4):268-274. Epub 2018 Aug 17.

Divisions of Diagnostic Imaging and Radiology, Children's National Health System, and The George Washington University School of Medicine, Washington, District of Columbia, USA.

Objectives: Chiari II malformation is a congenital abnormality characterized by a small posterior fossa with downward displacement of the hindbrain into the foramen magnum. Diffusion-weighted imaging (DWI) can be used to quantitatively characterize brain injury and physiological maturation. We aim to evaluate DWI parameters of the infra- and supratentorial brain in fetuses with Chiari II malformation.

Methods: MRI and US studies of 26 fetuses with Chiari II malformation and 15 controls were evaluated for the presence/severity of hydrocephalus, myelomeningocele level, and brain apparent diffusion coefficient (ADC) values. Cerebral flow impedance parameters (resistance and pulsatile indexes, resistance index [RI], and pulsatility index [PI] respectively) from concurrently preformed fetal US were also evaluated.

Results: Of the Chiari II group, 16 fetuses had hydrocephalus. There was increasing severity of lateral ventriculomegaly in the high compared to low spinal dysraphism subgroups. There were significant lower ADC values in the frontal (p = 0.01) and temporal lobes (p = 0.05) in Chiari II group compared with normal, regardless of the presence or severity of hydrocephalus. Fetuses with Chiari II showed significantly lower RI and PI values.

Conclusions: Abnormal ADC values indicate supratentorial microstructural changes in fetuses with Chiari II malformation. Further investigation of the role of diffusion imaging metrics in evaluating abnormal brain development, parenchymal damage and efficacy of fetal surgery is needed.
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http://dx.doi.org/10.1159/000490102DOI Listing
August 2019

Ultrasound and CT of the posterior fossa in neonates.

Handb Clin Neurol 2018 ;154:205-217

Division of Pediatric Radiology and Pediatric Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Medical Institutions, Baltimore, MD, United States. Electronic address:

Ultrasound, CT and MRI may all be used in the evaluation of the posterior fossa in neonates depending on the clinical scenario. Ultrasonography is particularly valuable for the evaluation of the neonatal brain because of the lack of ionizing radiation and the ability to perform exams at the bedside and, importantly, advancements in ultrasound technology now allow for diagnostic-quality imaging. While CT is still the initial imaging modality of choice in most neurologic emergencies, in the neonate, ultrasound is the first line in nontraumatic emergencies. The goal of this chapter is to discuss the ultrasound technique for evaluation of the cerebellum, to describe the normal sonographic and CT appearance of posterior fossa and to provide the classical findings of the most common cerebellar abnormalities. While ultrasound is able to accurately diagnosis a majority of cerebellar abnormalities in neonates, subsequent MR imaging remains essential to confirm findings and to detect associated abnormalities.
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http://dx.doi.org/10.1016/B978-0-444-63956-1.00012-6DOI Listing
September 2018

Neuroimaging Findings of Organic Acidemias and Aminoacidopathies.

Radiographics 2018 May-Jun;38(3):912-931

From the Division of Pediatric Radiology and Pediatric Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science (N.R., S.F.C., T.A.G.M.H., B.P.S.), and McKusick-Nathans Institute of Genetic Medicine, Department of Pediatrics (H.J.V.), The Johns Hopkins University School of Medicine, Charlotte R. Bloomberg Children's Center Bldg, Sheikh Zayed Tower, Room 4174, 1800 Orleans St, Baltimore, MD 21287-0842; Università degli Studi di Milano, Postgraduation School in Radiodiagnostics, Milan, Italy (S.F.C.); Department of Neurogenetics, Kennedy Krieger Institute, Baltimore, Md (H.J.V.); and Department of Pediatric Neurology, University Children's Hospital of Zurich, Zurich, Switzerland (E.B.).

Although individual cases of inherited metabolic disorders are rare, overall they account for a substantial number of disorders affecting the central nervous system. Organic acidemias and aminoacidopathies include a variety of inborn errors of metabolism that are caused by defects in the intermediary metabolic pathways of carbohydrates, amino acids, and fatty acid oxidation. These defects can lead to the abnormal accumulation of organic acids and amino acids in multiple organs, including the brain. Early diagnosis is mandatory to initiate therapy and prevent permanent long-term neurologic impairments or death. Neuroimaging findings can be nonspecific, and metabolism- and genetics-based laboratory investigations are needed to confirm the diagnosis. However, neuroimaging has a key role in guiding the diagnostic workup. The findings at conventional and advanced magnetic resonance imaging may suggest the correct diagnosis, help narrow the differential diagnosis, and consequently facilitate early initiation of targeted metabolism- and genetics-based laboratory investigations and treatment. Neuroimaging may be especially helpful for distinguishing organic acidemias and aminoacidopathies from other more common diseases with similar manifestations, such as hypoxic-ischemic injury and neonatal sepsis. Therefore, it is important that radiologists, neuroradiologists, pediatric neuroradiologists, and clinicians are familiar with the neuroimaging findings of organic acidemias and aminoacidopathies. RSNA, 2018.
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http://dx.doi.org/10.1148/rg.2018170042DOI Listing
October 2018

ACR Appropriateness Criteria Headache-Child.

J Am Coll Radiol 2018 May;15(5S):S78-S90

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

Headaches in children are not uncommon and have various causes. Proper neuroimaging of these children is very specific to the headache type. Care must be taken to choose and perform the most appropriate initial imaging examination in order to maximize the ability to properly determine the cause with minimum risk to the child. This evidence-based report discusses the different headache types in children and provides appropriate guidelines for imaging these children. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2018.03.017DOI Listing
May 2018

Spontaneous epidural hematoma secondary to bone infarction in sickle cell anemia: case report.

J Neurosurg Pediatr 2018 07 20;22(1):18-21. Epub 2018 Apr 20.

Departments of1Radiology and.

Spontaneous epidural hematoma (EDH) is a rare occurrence in patients with sickle cell disease, with a small number of cases reported. Appropriate diagnosis is critical, because rapid neurosurgical intervention may be required. This unique case illustrates clinical and MRI features of an 18-year-old woman presenting with a headache and subsequent progression to severe focal neurological symptoms. Imaging demonstrated a large EDH of mixed signal characteristics and underlying calvarial infarction, requiring emergency decompression and evacuation. A second companion case is also presented. The authors discuss proposed pathophysiology of the formation of EDHs in sickle cell anemia.
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http://dx.doi.org/10.3171/2018.1.PEDS17407DOI Listing
July 2018

The Child With Macrocephaly: Differential Diagnosis and Neuroimaging Findings.

AJR Am J Roentgenol 2018 Apr 22;210(4):848-859. Epub 2018 Feb 22.

3 Russell H. Morgan Department of Radiology and Radiological Science, Division of Pediatric Radiology and Pediatric Neuroradiology, Johns Hopkins University School of Medicine, The Johns Hopkins Hospital, 1800 Orleans St, Zayed Tower, Rm 4174, Baltimore, MD 21287.

Objective: The purpose of this article is to offer a systematic approach to the imaging of children with macrocephaly and to illustrate key neuroimaging features of common and rare but important disorders.

Conclusion: Macrocephaly is a common clinical finding in children. Increased volume of one of the intracranial compartments can enlarge the head either prenatally or postnatally while the cranial sutures are open. Imaging plays a central role in establishing a diagnosis and guiding management.
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http://dx.doi.org/10.2214/AJR.17.18693DOI Listing
April 2018

Bottom-of-sulcus focal cortical dysplasia presenting as epilepsia partialis continua multimodality characterization including 7T MRI.

Childs Nerv Syst 2018 06 14;34(6):1267-1269. Epub 2018 Feb 14.

Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 1800 Orleans Street, Zayed Tower, Room 4174, Baltimore, MD, 21287, USA.

Introduction: Bottom-of-sulcus focal cortical dysplasias are an under recognized, surgically treatable cause of focal epilepsy. Resection can dramatically reduce the seizure burden for children with refractory epilepsy, or eliminate seizures altogether.

Material And Methods: We report the case and present the results of multimodality evaluation of a 15-year-old young man who presented with long-standing partial epilepsy affecting his right leg, which over the years became refractory to therapy.

Results: High-resolution 3T MRI images acquired as a dedicated epilepsyprotocol were initially interpreted as unremarkable. On further review by an experienced specialist aware of clinical and electroencephalographic findings, a subtle focal cortical dysplasia was identified at the bottom of a sulcus near the medial aspect of the left precentral gyrus. After confirmation of the extent of the lesion with PET and ultra-high field 7T MRI, the patient underwent cortical mapping and focal resection and remains free of seizures.

Coclusions: This case emphasizes the need for a multidisciplinary approach to the evaluation of refractory focal epilepsy in children and highlights the potential role of ultra-high field 7T MRI in identifying the often subtle causative anatomic abnormalities.
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http://dx.doi.org/10.1007/s00381-018-3749-2DOI Listing
June 2018

Stereotactic laser amygdalohippocampotomy for mesial temporal lobe epilepsy.

Ann Neurol 2018 03 3;83(3):575-587. Epub 2018 Mar 3.

Department of Neurology, Emory University School of Medicine, Atlanta, GA.

Objective: To evaluate the outcomes 1 year and longer following stereotactic laser amygdalohippocampotomy for mesial temporal lobe epilepsy in a large series of patients treated over a 5-year period since introduction of this novel technique.

Methods: Surgical outcomes of a consecutive series of 58 patients with mesial temporal lobe epilepsy who underwent the surgery at our institution with at least 12 months of follow-up were retrospectively evaluated. A subgroup analysis was performed comparing patients with and without mesial temporal sclerosis.

Results: One year following stereotactic laser amygdalohippocampotomy, 53.4% (95% confidence interval [CI] = 40.8-65.7%) of all patients were free of disabling seizures (Engel I). Three of 9 patients became seizure-free following repeat ablation. Subgroup analysis showed that 60.5% (95% CI = 45.6-73.7%) of patients with mesial temporal sclerosis were free of disabling seizures as compared to 33.3% (95% CI = 15.0-58.5%) of patients without mesial temporal sclerosis. Quality of Life in Epilepsy-31 scores significantly improved at the group level, few procedure-related complications were observed, and verbal memory outcome was better than historical open resection data.

Interpretation: In an unselected consecutive series of patients, stereotactic laser amygdalohippocampotomy yielded seizure-free rates for patients with mesial temporal lobe epilepsy lower than, but comparable to, the outcomes typically associated with open temporal lobe surgery. Analogous to results from open surgery, patients without mesial temporal sclerosis fared less well. This novel procedure is an effective minimally invasive alternative to resective surgery. In the minority of patients not free of disabling seizures, laser ablation presents no barrier to additional open surgery. Ann Neurol 2018;83:575-587.
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http://dx.doi.org/10.1002/ana.25180DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5877322PMC
March 2018

Dysplasia and overgrowth: magnetic resonance imaging of pediatric brain abnormalities secondary to alterations in the mechanistic target of rapamycin pathway.

Neuroradiology 2018 Feb 26;60(2):137-150. Epub 2017 Dec 26.

Division of Pediatric Radiology and Pediatric Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 1800 Orleans Street, Zayed 4174, Baltimore, MD, 21287, USA.

The current classification of malformations of cortical development is based on the type of disrupted embryological process (cell proliferation, migration, or cortical organization/post-migrational development) and the resulting morphological anomalous pattern of findings. An ideal classification would include knowledge of biological pathways. It has recently been demonstrated that alterations affecting the mechanistic target of rapamycin (mTOR) signaling pathway result in diverse abnormalities such as dysplastic megalencephaly, hemimegalencephaly, ganglioglioma, dysplastic cerebellar gangliocytoma, focal cortical dysplasia type IIb, and brain lesions associated with tuberous sclerosis. We review the neuroimaging findings in brain abnormalities related to alterations in the mTOR pathway, following the emerging trend from morphology towards genetics in the classification of malformations of cortical development. This approach improves the understanding of anomalous brain development and allows precise diagnosis and potentially targeted therapies that may regulate mTOR pathway function.
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http://dx.doi.org/10.1007/s00234-017-1961-5DOI Listing
February 2018

Orbital infantile hemangioma and rhabdomyosarcoma in children: differentiation using diffusion-weighted magnetic resonance imaging.

J AAPOS 2018 02 4;22(1):27-31. Epub 2017 Nov 4.

Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russel H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Purpose: To evaluate differences in magnetic resonance imaging (MRI) appearance between infantile hemangiomas and rhabdomyosarcomas of the orbit in pediatric patients using diffusion-weighted imaging.

Methods: A multicenter retrospective review of MRIs of pediatric patients with infantile hemangiomas and rhabdomyosarcomas of the orbit was performed. MRI examinations from a total of 21 patients with infantile hemangiomas and 12 patients with rhabdomyosarcomas of the orbit were independently reviewed by two subspecialty board-certified neuroradiologists masked to the diagnosis. A freehand region of interest was placed in the mass to obtain the mean apparent diffusion coefficient (ADC) value of the mass as well as within the medulla to obtain a ratio of the ADC mass to the medulla. A t test was used to compare mean ADC and ADC ratios between the two groups. Receiver operating characteristic analysis was performed to determine ADC value and ADC ratio thresholds for differentiation of infantile hemangioma and rhabdomyosarcoma.

Results: There was a statistically significant difference in the mean ADC value of infantile hemangiomas compared to rhabdomyosarcomas (1527 × 10 mm/s vs 782 × 10 mm/s; P = 0.0001) and the ADC ratio of the lesion to the medulla (1.77 vs 0.92; P = 0.0001). An ADC threshold of <1159 × 10 mm/sec and an ADC ratio of <1.38 differentiated rhabdomyosarcoma from infantile hemangioma (sensitivity 100% and 100%; specificity 100% and 100%) with area under the curve of 1.0 and 1.0, respectively.

Conclusions: In conjunction with conventional MRI sequences, ADC values obtained from diffusion-weighted MRI are useful to differentiate orbital infantile hemangiomas from rhabdomyosarcomas in pediatric patients.
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http://dx.doi.org/10.1016/j.jaapos.2017.09.002DOI Listing
February 2018