Mechanical disorders of the cervicocerebral circulation in children and young adults.

Mechanical disorders of the cervicocerebral circulation (MDCC) are conditions in which neurological symptoms result from a disturbance of cerebral blood flow attributable to external mechanical forces exerted on extracranial blood vessels by adjacent musculoskeletal structures during head movement that is presumably within a physiological range. The disease spectrum includes bow hunter's syndrome, carotid-type Eagle syndrome, and various dynamic venous compression syndromes. These conditions have distinct phenotypes in children which differ from those expressed in older adults. In contemporary practice, recognition and diagnostic evaluation is the domain of the neuroendovascular specialist. The diagnostic evaluation of MDCC involves significant technical nuance that can be critical to directing appropriate management, particularly in children. This report aims to provide a comprehensive overview of the pathophysiology, anatomical patterns, diagnosis, and treatment for the full spectrum of MDCC that is commonly encountered in clinical practice.

Pediatric spinal cord diffuse midline glioma, H3 K27-altered with intracranial and spinal leptomeningeal spread: A case report.

Primary spinal cord high-grade gliomas, including those histologically identified as glioblastoma (GBM), are a rare entity in the pediatric population but should be considered in the differential diagnosis of intramedullary lesions. Pediatric spinal cord high-grade gliomas have an aggressive course with poor prognosis. The aim of this case report is to present a 15-year-old female adolescent with histopathologically confirmed spinal cord GBM with H3F3A K27 M mutation consistent with a diffuse midline glioma (DMG), H3 K27-altered, CNS WHO grade 4 with leptomeningeal seeding on initial presentation. As imaging features of H3 K27-altered DMGs are non-specific and may mimic more frequently encountered neoplastic diseases as well as demyelinating disorders, severe neurological deficits at presentation with short duration, rapid progression, and early leptomeningeal seeding should however raise the suspicion for a pediatric-type diffuse high-grade glioma like DMG, H3 K27-altered.

A proposed framework for cerebral venous congestion.

BACKGROUND: While venous congestion in the peripheral vasculature has been described and accepted, intracranial venous congestion remains poorly understood. The characteristics, pathophysiology, and management of cerebral venous stasis, venous hypertension and venous congestion remain controversial, and a unifying conceptual schema is absent. The cerebral venous and lymphatic systems are part of a complex and dynamic interaction between the intracranial compartments, with interplay between the parenchyma, veins, arteries, cerebrospinal fluid, and recently characterized lymphatic-like systems in the brain. Each component contributes towards intracranial pressure, occupying space within the fixed calvarial volume. This article proposes a framework to consider conditions resulting in brain and neck venous congestion, and seeks to expedite further study of cerebral venous diagnoses, mechanisms, symptomatology, and treatments. METHODS: A multi-institution retrospective review was performed to identify unique patient cases, complemented with a published case series to assess a spectrum of disease states with components of venous congestion affecting the brain. These diseases were organized according to anatomical location and purported mechanisms. Outcomes of treatments were also analyzed. Illustrative cases were identified in the venous treatment databases of the authors. CONCLUSION: This framework is the first clinically structured description of venous pathologies resulting in intracranial venous and cerebrospinal fluid hypertension. Our proposed system highlights unique clinical symptoms and features critical for appropriate diagnostic work-up and potential treatment. This novel schema allows clinicians effectively to approach cases of intracranial hypertension secondary to venous etiologies, and furthermore provides a framework by which researchers can better understand this developing area of cerebrovascular disease.

Conventional and advanced MR imaging findings in a cohort of pathology-proven dermoid cysts of the pediatric scalp and skull.

BACKGROUND: In the pediatric population, dermoid cysts are among the most frequent lesions of the scalp and skull. Imaging plays a key role in characterizing scalp and skull lesions in order to narrow the differential diagnoses. In general, dermoids are described as heterogeneous T1-/T2-hypo- to hyperintense lesions on magnetic resonance imaging. METHODS: The goal of this retrospective study is to evaluate the diffusion weighted imaging findings while reviewing the conventional T1-/T2-/T1+C-weighted MR characteristics in a pathology-proven series of 14 dermoids of the pediatric scalp and skull. RESULTS: In our pediatric cohort (eight boys, six girls, age range 3-95 months), half of the dermoids were homogeneous T1-hypointense and homogeneous T2-hyperintense. We found a mixture of restricted (45.5%) and increased diffusion (54.5%) in dermoids. The vast majority of dermoids (91.7%) showed rim enhancement. Most dermoids (57.1%) were located at the midline and adjacent to one of its sutures. CONCLUSIONS: This study suggests that dermoids may have more variable imaging appearances than hitherto assumed and are frequently seen in close proximity or adjacent to the anterior fontanelle.

Possible additional MRI markers for critical brain swelling with increased intracranial pressure in children?

A retrospective study was performed evaluating the volume and T2/T1 signal intensity of orbital fat in five children diagnosed with severe global brain swelling and confirmed cessation of cerebral perfusion. Imaging showed a volume increase (five of five) and a heterogeneous T2-hypointensity of orbital fat (four of five). This preliminary study suggests that swelling and T2-hypointensity of orbital fat may be a marker of global brain swelling and/or increased intracranial pressure.

Bedside contrast-enhanced ultrasound diagnosing cessation of cerebral circulation in a neonate: A novel bedside diagnostic tool.

Imaging diagnosis of brain death is performed with either four-vessel cerebral angiography or radionuclide cerebral blood flow studies. Unfortunately, timely performance of either study at a critically ill period is not only cumbersome but not feasible in many cases. We present a case of a 6-month-old male three hours status post-cardiac arrest of unknown etiology who underwent contrast-enhanced ultrasound (CEUS) for diagnosis of near absent perfusion, or near brain death. The patient passed away 30 minutes after the exam and clinical diagnosis of brain death was confirmed. The case report highlights the utility of CEUS for diagnosis of brain death. This can have significant clinical implications in neonates who may not be eligible for commonly used, cumbersome radiologic studies for diagnosis of brain death.

Intracranial dural chondroma in a child-conventional and advanced neuroimaging characteristics and differential diagnosis.

Intracranial chondromas are rare tumors, especially in the pediatric population. We describe the conventional and advanced neuroimaging characteristics of this rare convexity dura-based chondroma in a young adolescent. In particular we demonstrate that diffusion-weighted imaging (DWI) facilitates differentiation between a dura-based chondroma and the more frequent classical meningioma. Chondromas are typically DWI hypointense with high apparent diffusion coefficient (ADC) values while meningiomas are typically DWI hyperintense with low ADC values. We also discuss the relevant additional differential diagnoses of dura based focal lesions for the pediatric population as well as the diagnostic significance of additional imaging modalities, including computed tomography, magnetic resonance imaging and cerebral angiography.

Blake's pouch cyst in children: Atypical clinical presentation.

Blake's pouch cyst is a posterior fossa cystic malformation characterized by a infracerebellar cyst, absence of communication between the fourth ventricle and the subarachnoid space, and tetraventricular hydrocephalus. Children with Blake's pouch cyst typically present with macrocephaly due to hydrocephalus during the neonatal period or infancy. Atypical presentation is, however, possible. Here we present clinical and neuroimaging findings, as well as management and outcome, of an 18-month-old girl with atypical presentation of Blake's pouch cyst characterized by cerebellar ataxia. Familiarity with the neuroimaging findings of Blake's pouch cyst and differentiation between Blake's pouch cyst and other posterior fossa cystic malformations is important in terms of diagnosis, management, prognosis, and counseling of the affected families.

Radiological findings of abnormalities associated with congenital Zika virus infection: conclusions from World Radiology Day 2016

[ABSTRACT]. In July 2015, Brazil reported an association between Zika virus infection and Guillain-Barré syndrome, and then in October 2015, between Zika and microcephaly. Most countries and territories in the Region of the Americas were later affected by the virus, creating a public health emergency. Each year, the Pan American Health Organization (PAHO), Regional Office of the World Health Organization commemorates World Radiology Day (WRD), which highlights the role of radiology in public health. In 2016, PAHO devoted its WRD efforts to the Zika infection. Experts and partners presented and discussed the various radiological findings of Zika infection, the crucial role of obstetric ultrasound in the screening and monitoring of abnormalities associated with confirmed Zika virus infection, and the appropriateness of utilizing other neuroimaging technologies to study brain abnormalities in neonates and infants with prenatal Zika virus infection. The conclusions of WRD 2016 recommend that upon confirmation, prenatal ultrasound be used as the main tool to investigate and monitor suspected cases, with subsequent multidisciplinary postnatal assessments that include neuropediatric clinical studies and relevant neuroimaging. Additionally, radiology technicians should be adequately trained and a quality assurance program should be implemented to ensure timely, safe, and accurate diagnosis.

[RESUMEN]. En julio del 2015, Brasil informó una asociación entre la infección por el virus del Zika y el síndrome de Guillain-Barré y luego, en octubre 2015, entre la infección por el virus del Zika y la microcefalia. Posteriormente, la mayoría de los países y territorios de la Región de las Américas se vio afectada por el virus, lo que dio lugar a una emergencia de salud pública. Todos los años, la Organización Panamericana de la Salud (OPS), la Oficina Regional de la Organización Mundial de la Salud, conmemora el Día Mundial de la Radiología, en el que se reconoce la importancia de la radiología en la salud pública. En el 2016, la OPS dedicó las actividades en el marco del Día Mundial de la Radiología a la infección por el virus del Zika. Diversos expertos y asociados presentaron los distintos signos radiográficos de la infección por el virus del Zika, y debatieron sobre esto, así como sobre el papel fundamental que desempeñan las ecografías obstétricas en el tamizaje y el seguimiento de las anomalías asociadas con la infección por el virus del Zika confirmada, y sobre la pertinencia de utilizar técnicas de neurodiagnóstico por imágenes para estudiar las anomalías cerebrales en recién nacidos y lactantes con infección por el virus del Zika prenatal. Las conclusiones del Día Mundial de la Radiología del 2016 recomiendan que, tras la confirmación de la infección, se utilicen las ecografías prenatales como herramienta principal para investigar y hacer un seguimiento de los casos presuntos, seguida por una evaluación posnatal multidisciplinaria en la que se incluyan estudios clínicos neuropediátricos y las técnicas de neurodiagnóstico por imágenes pertinentes. Además, se debe capacitar adecuadamente a los técnicos de radiología y se debe ejecutar un programa de garantía de la calidad para asegurar el diagnóstico oportuno, seguro y certero.

[RESUMO]. Em julho de 2015, o Brasil informou existir associação entre a infecção por vírus zika e síndrome de Guillain-Barré e, em outubro do mesmo ano, entre o vírus zika e microcefalia. A maioria de países e territórios na Região das Américas foi subsequentemente afetada pelo vírus, constituindo emergência de saúde pública. Todos os anos, a Organização Pan-Americana da Saúde (OPAS), Escritório Regional da Organização Mundial da Saúde (OMS) comemora o Dia Internacional da Radiologia destacando o papel da radiologia em saúde pública. Em 2016, a OPAS dedicou este dia à infecção por vírus zika. Especialistas e parceiros apresentaram e debateram os diversos achados radiológicos na infecção por vírus zika, o papel fundamental do exame de ultrassom obstétrico na detecção precoce e monitoramento de anomalias associadas à infecção confirmada e quando convém usar outras técnicas de neuroimagem para estudar as anomalias cerebrais nos recém-nascidos e lactentes com infecção pré-natal. O painel do Dia Internacional da Radiologia 2016 recomenda que, com a confirmação da infecção, o ultrassom pré-natal seja o principal método usado para investigar e monitorar os casos suspeitos, com subsequente avaliação pós-natal multidisciplinar incluindo estudos clínicos neuropediátricos e exames de neuroimagem. Além disso, o pessoal técnico em radiologia deve ser adequadamente capacitado e implantado um programa de garantia da qualidade para assegurar o diagnóstico preciso, seguro e oportuno.

Back pain and scoliosis in children: When to image, what to consider.

Back pain and scoliosis in children most commonly present as benign and self-limited entities. However, persistent back pain and/or progressive scoliosis should always be taken seriously in children. Dedicated diagnostic work-up should exclude etiologies that may result in significant morbidity. Clinical evaluation and management require a comprehensive history and physical and neurological examination. A correct imaging approach is important to define a clear diagnosis and should be reserved for children with persistent symptoms or concerning clinical and laboratory findings. This article reviews the role of different imaging techniques in the diagnostic approach to back pain and scoliosis, and offers a comprehensive review of the main imaging findings associated with common and uncommon causes of back pain and scoliosis in the pediatric population.

Ventricular pseudodiverticula from intraparenchymal cerebrospinal fluid dissection secondary to high-grade obstructive hydrocephalus in children: magnetic resonance imaging findings.

We report on a series of three children who presented with a focal cerebrospinal fluid collection within the periventricular white matter of the temporal and occipital lobes in the setting of high-grade obstructive hydrocephalus. Magnetic resonance imaging showed a focal defect within the ventricular wall associated with leakage of cerebrospinal fluid into the adjacent white matter. The white matter tracts appeared primarily displaced. This entity should be referred to as ventricular pseudodiverticulum, not lined by ependymal cells, in contrast to a true ventricular diverticulum in which the cerebrospinal fluid is contained by a focal outpouching of the intact ventricular wall lined by a dilated and prolapsed layer of ependymal cells. Correct interpretation and classification of the findings may be helpful in predicting prognosis and outcome.

Acute brain injury following illicit drug abuse in adolescent and young adult patients: spectrum of neuroimaging findings.

The use of illicit drugs is currently a major medical problem among adolescents. Several illicit drugs have a high abuse potential and can be neurotoxic causing high morbidity and mortality. The clinical manifestation of adolescents with acute drug-induced neurotoxicity is often characterized by non-specific symptoms and findings. Early diagnosis is important to prevent death and permanent long-term neurological impairments. We report on clinical and neuroimaging findings in five adolescents with acute brain imaging following illicit drug intoxication to highlight the role of neuroimaging findings in the diagnostic work-up of pediatric acute drug-induced neurotoxicity. Our patients reveal two main neuroimaging patterns of brain injury: diffuse symmetric subcortical white matter injury with preferential cerebellar involvement (leukoencephalopathy pattern) or multiple foci of ischemic infarctions in a non-arterial territory distribution (ischemic pattern). Familiarity with these two neuroimaging patterns of findings in the evaluation of magnetic resonance imaging studies in adolescents with acutely altered mental status may suggest the correct diagnosis, narrow the differential diagnosis, and consequently allow early initiation of targeted laboratory investigations and treatment, potentially improving outcome.

Radiological findings of abnormalities associated with congenital Zika virus infection: conclusions from World Radiology Day 2016 / Resultados radiológicos de las anomalías asociadas con la infección congénita por el virus del Zika: conclusiones del Día Mundial de la Radiología del 2016 / Achados radiológicos de anomalias associadas à infecção congênita por vírus zika: conclusões do Dia Internacional da Radiologia 2016

ABSTRACT In July 2015, Brazil reported an association between Zika virus infection and Guillain-Barré syndrome, and then in October 2015, between Zika and microcephaly. Most countries and territories in the Region of the Americas were later affected by the virus, creating a public health emergency. Each year, the Pan American Health Organization (PAHO), Regional Office of the World Health Organization commemorates World Radiology Day (WRD), which highlights the role of radiology in public health. In 2016, PAHO devoted its WRD efforts to the Zika infection. Experts and partners presented and discussed the various radiological findings of Zika infection, the crucial role of obstetric ultrasound in the screening and monitoring of abnormalities associated with confirmed Zika virus infection, and the appropriateness of utilizing other neuroimaging technologies to study brain abnormalities in neonates and infants with prenatal Zika virus infection. The conclusions of WRD 2016 recommend that upon confirmation, prenatal ultrasound be used as the main tool to investigate and monitor suspected cases, with subsequent multidisciplinary postnatal assessments that include neuropediatric clinical studies and relevant neuroimaging. Additionally, radiology technicians should be adequately trained and a quality assurance program should be implemented to ensure timely, safe, and accurate diagnosis.

RESUMEN En julio del 2015, Brasil informó una asociación entre la infección por el virus del Zika y el síndrome de Guillain-Barré y luego, en octubre 2015, entre la infección por el virus del Zika y la microcefalia. Posteriormente, la mayoría de los países y territorios de la Región de las Américas se vio afectada por el virus, lo que dio lugar a una emergencia de salud pública. Todos los años, la Organización Panamericana de la Salud (OPS), la Oficina Regional de la Organización Mundial de la Salud, conmemora el Día Mundial de la Radiología, en el que se reconoce la importancia de la radiología en la salud pública. En el 2016, la OPS dedicó las actividades en el marco del Día Mundial de la Radiología a la infección por el virus del Zika. Diversos expertos y asociados presentaron los distintos signos radiográficos de la infección por el virus del Zika, y debatieron sobre esto, así como sobre el papel fundamental que desempeñan las ecografías obstétricas en el tamizaje y el seguimiento de las anomalías asociadas con la infección por el virus del Zika confirmada, y sobre la pertinencia de utilizar técnicas de neurodiagnóstico por imágenes para estudiar las anomalías cerebrales en recién nacidos y lactantes con infección por el virus del Zika prenatal. Las conclusiones del Día Mundial de la Radiología del 2016 recomiendan que, tras la confirmación de la infección, se utilicen las ecografías prenatales como herramienta principal para investigar y hacer un seguimiento de los casos presuntos, seguida por una evaluación posnatal multidisciplinaria en la que se incluyan estudios clínicos neuropediátricos y las técnicas de neurodiagnóstico por imágenes pertinentes. Además, se debe capacitar adecuadamente a los técnicos de radiología y se debe ejecutar un programa de garantía de la calidad para asegurar el diagnóstico oportuno, seguro y certero.

RESUMO Em julho de 2015, o Brasil informou existir associação entre a infecção por vírus zika e síndrome de Guillain-Barré e, em outubro do mesmo ano, entre o vírus zika e microcefalia. A maioria de países e territórios na Região das Américas foi subsequentemente afetada pelo vírus, constituindo emergência de saúde pública. Todos os anos, a Organização Pan-Americana da Saúde (OPAS), Escritório Regional da Organização Mundial da Saúde (OMS) comemora o Dia Internacional da Radiologia destacando o papel da radiologia em saúde pública. Em 2016, a OPAS dedicou este dia à infecção por vírus zika. Especialistas e parceiros apresentaram e debateram os diversos achados radiológicos na infecção por vírus zika, o papel fundamental do exame de ultrassom obstétrico na detecção precoce e monitoramento de anomalias associadas à infecção confirmada e quando convém usar outras técnicas de neuroimagem para estudar as anomalias cerebrais nos recém-nascidos e lactentes com infecção pré-natal. O painel do Dia Internacional da Radiologia 2016 recomenda que, com a confirmação da infecção, o ultrassom pré-natal seja o principal método usado para investigar e monitorar os casos suspeitos, com subsequente avaliação pós-natal multidisciplinar incluindo estudos clínicos neuropediátricos e exames de neuroimagem. Além disso, o pessoal técnico em radiologia deve ser adequadamente capacitado e implantado um programa de garantia da qualidade para assegurar o diagnóstico preciso, seguro e oportuno.

Neuroimaging findings in acute pediatric diabetic ketoacidosis.

Diabetic ketoacidosis (DKA) is a state of severe insulin deficiency and a serious complication in children with diabetes mellitus type 1. In a small number of children, DKA is complicated by injury of the central nervous system. These children have a significant mortality and high long-term neurological morbidity. Cerebral edema is the most common neuroimaging finding in children with DKA and may cause brain herniation. Ischemic or hemorrhagic stroke during the acute DKA episode is less common and accounts for approximately 10% of intracerebral complications of DKA. Here we present the neuroimaging findings of two children with DKA and brain injury. Familiarity with the spectrum of neuroimaging findings seen in pediatric DKA is important to allow early detection as well as initiation of therapy and, hence, prevent complications of the central nervous system.

Chiari type 1 malformation, corpus callosum agenesis and patent craniopharyngeal canal in an 11-year-old boy.

We describe the neuroimaging findings of an 11-year-old boy who presented with mild occipital headache and precocious puberty. This child was found to have a combination of various midline anomalies including a Chiari type 1 malformation, corpus callosum agenesis and patent craniopharyngeal canal with adjacent intracranial dermoid cyst.

Cerebral sinovenous thrombosis in a child with Crohn's disease, otitis media, and meningitis.

Pediatric cerebral sinovenous thrombosis (CSVT) is associated with high morbidity and mortality. Severe long-term sequelae are reported in up to 48% of children. The most frequent location of CSVT in children is the superficial venous system. We present the neuroimaging findings using both computed tomography and magnetic resonance imaging (MRI) in a 10-year-old child with extensive superficial CSVT. Our report aims to stress the importance of awareness of risk factors in suspecting and rapidly diagnosing CSVT. The application of targeted conventional and advanced MRI sequences is the diagnostic tool of choice in children at risk of or with clinically suspected CSVT.

Diffusion tensor imaging in pediatric Chiari type I malformation.

AIM: Chiari type I malformation (C1M) may be symptomatic or asymptomatic as an incidental finding. In this retrospective study, we applied diffusion tensor imaging (DTI) to study the brainstem and cerebellar white matter tracts in C1M. METHOD: Diffusion tensor imaging (DTI) data were acquired on a 1.5T MR-scanner using balanced pairs of diffusion gradients along 20 non-collinear directions. Measurements from regions of interest in each pontine corticospinal tract, medial leminscus, and middle cerebellar peduncle (MCP) and in the lower brainstem were obtained for fractional anisotropy and mean, axial, and radial diffusivity. Values in symptomatic and asymptomatic children, and children with and without hydromyelia were compared using analysis of variance. RESULTS: Fifteen children with C1M (10 males, five females; six symptomatic [four with hydromyelia] and nine asymptomatic) were included. Median age was 6 years 5 months (range 2y 10mo-15y 4mo). No significant differences in DTI scalars were found in the lower brainstem. In both MCPs, axial diffusivity values were lower in symptomatic than in asymptomatic children (p=0.049 and p=0.035 respectively) and higher in children with hydromyelia versus without hydromyelia (p=0.018 and p=0.006 respectively). In the left MCP, mean diffusivity values were lower in symptomatic than in asymptomatic children (p=0.047). INTERPRETATION: Our results show that microstructural tissue alterations may be present in C1M. Additionally, our study suggests a specific role for the MCPs in C1M. Further large-scale studies are warranted.

Pediatric hemiplegic migraine: role of multiple MRI techniques in evaluation of reversible hypoperfusion.

BACKGROUND: Hemiplegic migraine (HM) is a rare type of migraine with aura that involves motor weakness. Data on conventional and advanced neuroimaging findings during prolonged attacks of HM are limited, particularly in children. CASE: A 13-year-old-female with a history of migraine had a typical attack of HM characterized by right-sided hemiplegia, deterioration of vigilance and paraphasia. MRI performed 3 hours after hemiplegia onset revealed normal diffusion tensor imaging (DTI) sequences, but perfusion weighted imaging (PWI) showed a large area of hypoperfusion within the left cerebral hemisphere and susceptibility weighted imaging (SWI) demonstrated a matching area with prominent, hypointense draining sulcal veins. Magnetic resonance angiography (MRA) revealed subtle narrowing of the left middle cerebral artery. The neuroimaging abnormalities completely resolved 24 hours after the attack onset. CONCLUSION: Multiple conventional and advanced MRI techniques including SWI play a key role in an HM attack to (1) exclude acute arterial ischemic stroke and (2) further understand the pathophysiology of HM.