Advances in Imaging Modalities for Pediatric Brain and Spinal Cord Tumors.

BACKGROUND: Neuroimaging has evolved from anatomical imaging toward a multi-modality comprehensive anatomical and functional imaging in the past decades, important functional data like perfusion-weighted imaging, permeability imaging, diffusion-weighted imaging (DWI), and diffusion tensor imaging (DTI), tractography, metabolic imaging, connectomics, event-related functional imaging, resting state functional imaging, and much more is now being offered. SUMMARY: Precision diagnostics has proven to be essential for precision treatment. Many minimal invasive techniques have been developed, taking advantage of digital subtraction angiography and interventional neuroradiology. Furthermore, intraoperative CT and/or MRI and more recently MR-guided focused ultrasound have complemented the diagnostic and therapeutic armamentarium. KEY MESSAGES: In the current manuscript, we discuss standard imaging sequences including advanced techniques like DWI, DTI, susceptibility-weighted imaging, and 1H magnetic resonance spectroscopy, various perfusion weighted imaging approaches including arterial spin labeling, dynamic contrast enhanced imaging, and dynamic susceptibility contrast imaging. Pre-, intra, and postoperative surgical imaging including visualize imaging will be discussed. The value of connectomics will be presented for its value in neuro-oncology. Minimal invasive therapeutic possibilities of interventional neuroradiology and image-guided laser ablation and MR-guided high-intensity-focused ultrasound will be presented for treatment of pediatric brain and spinal cord tumors. Finally, a comprehensive review of spinal cord tumors and matching neuropathology has been included.

Another Piece of the Puzzle of Anomalous Connectivity in Joubert's Syndrome.

We report on the conventional and diffusion tensor imaging (DTI) findings of a 2-year-old child with clinical presentation of Joubert's Syndrome (JS) and brainstem structural abnormalities as depicted by neuroimaging.Conventional magnetic resonance imaging (MRI) showed a "molar tooth" configuration of the brainstem. A band-like formation coursing in an apparent axial plane anterior to the interpeduncular fossa was noted and appeared to partially cover the interpeduncular fossa.DTI maps and three-dimensional (3D) tractography demonstrated a prominent red-encoded white matter bundle anterior to the midbrain. Probable aberrant course of the bilateral corticospinal tracts (CST) was also depicted. Absence of the decussation of the superior cerebellar peduncles and elongated thickened, horizontal superior cerebellar peduncle (SCP) reflecting the molar tooth sign were also shown.Our report and the review of the published cases suggest that DTI and tractography may be very helpful to differentiate between interpeduncular heterotopias and similarly located white matter bundles corroborating the underlying etiology of axonal guidance disorders in the complex group of ciliopathies including JS. Our case represents an important additional puzzle piece to explore the variability of these ciliopathies.

Radiologic and clinical outcome of isolated fourth ventricle following post-hemorrhagic hydrocephalus in children.

PURPOSE: Few studies report radiologic and clinical outcome of post-hemorrhagic isolated fourth ventricle (IFV) with focus on surgical versus conservative management in neonates and children. Our aim is to investigate differences in radiological and clinical findings of IFV between patients who had surgical intervention versus patients who were treated conservatively. METHODS: A retrospective analysis of patients diagnosed with IFV was performed. Data included demographics, clinical exam findings, surgical history, and imaging findings (dilated FV extent, supratentorial ventricle dilation, brainstem and cerebellar deformity, tectal plate elevation, basal cistern and cerebellar hemisphere effacement, posterior fossa upward/downward herniation). RESULTS: Sixty-four (30 females) patients were included. Prematurity was 94% with 90% being < 28 weeks of gestation. Mean age at first ventricular shunt was 3.6 (range 1-19); at diagnosis of IFV, post-lateral ventricular shunting was 26.2 (1-173) months. Conservatively treated patients were 87.5% versus 12.5% treated with FV shunt/endoscopic fenestration. Severe FV dilation (41%), severe deformity of brainstem (39%) and cerebellum (47%) were noted at initial diagnosis and stable findings (34%, 47%, and 52%, respectively) were seen at last follow-up imaging. FV dilation (p = 0.0001) and upward herniation (p = 0.01) showed significant differences between surgery versus conservative management. No other radiologic or clinical outcome parameters were different between two groups. CONCLUSION: Only radiologic outcome results showed stable or normal FV dilation and stable or decreased upward herniation in the surgically treated group.

A descriptive neuroimaging study of retinoblastoma in children: magnetic resonance imaging features.

Purpose: Retinoblastoma (RB) is the most common intraocular malignancy of childhood. Magnetic resonance imaging (MRI) is essential for initial diagnosis, tumour extension, staging, and treatment planning of RB. Awareness of neuro-imaging findings and determining local extent are essential for early diagnosis and therapy guidance. The purpose of this study is to evaluate and to provide a detailed list of neuroimaging features of RB to improve the diagnostic work-up of children with RB. Material and methods: Retrospective review was performed among children with confirmed RB diagnosis. MRI features were identified to evaluate: 1) growth pattern; 2) intraocular extension; 3) extraocular extension; 4) central nervous system disease; 5) conventional MRI characteristics of the RB lesions; and 6) DWI and ADC characteristics. These features were compared between unilateral and bilateral RB lesions. Results: Twenty-four children (male/female: 18/6) were included in this study. The mean age at the time of diagnosis was 14.7 (11.4) months. In total, 34 RB lesions (bilateral = 18) were evaluated for the study. The most common features on MRI were: 1) endophytic RB lesion (50%); 2) subretinal haemorrhage (38%); 3) scleral involvement (3%); 4) leptomeningeal disease (12%); 5) contrast enhancement (97%); and 6) restricted diffusion (88%). The mean ADC value was 0.64 (0.15) × 10-3 mm2/s. Choroidal invasion (p = 0.05) and scleral involvement (p = 0.04) were significantly higher for bilateral RB lesions. Conclusions: Contrast enhancement and restricted diffusion are the most common neuroimaging features of RB. Choroidal invasion and scleral involvement are more frequently seen in bilateral disease.

Pulmonary manifestations and outcomes in paediatric ANCA-associated vasculitis: a single-centre experience.

OBJECTIVES: ANCA-associated vasculitis (AAV) usually involves the renal and respiratory systems, but the paediatric literature on pulmonary manifestations and outcomes is limited. We aimed to describe pulmonary manifestations and outcomes after therapy in a cohort of paediatric AAV (pAAV) patients. METHODS: A retrospective chart review of all patients <19 years presenting to our institution with AAV between 1/2008 and 2/2018 was conducted. Patient demographics, clinical presentation, diagnostic testing, therapy and pulmonary outcomes over the first 3 years after presentation were evaluated. RESULTS: A total of 38 patients were included; all had ANCA positivity by immunofluorescence. A total of 23 had microscopic polyangiitis (MPA), 13 had granulomatosis with polyangiitis and 2 had eosinophilic granulomatosis with polyangiitis. A total of 30 (79%) had pulmonary manifestations, with cough (73%) and pulmonary haemorrhage (67%) being the most common. Abnormalities were noted in 82% of chest CT scans reviewed, with nodules and ground-glass opacities being the most common. At 6, 12 and 36 months follow-up, respectively, 61.8%, 39.4% and 29% of patients continued to show pulmonary manifestations. Five MPA patients with re-haemorrhage are described in detail. CONCLUSION: MPA was more common than granulomatosis with polyangiitis, with pulmonary involvement being common in both. MPA patients had more severe pulmonary manifestations. Chest CT revealed abnormal findings in a majority of cases. A subgroup of young MPA patients experienced repeat pulmonary haemorrhage. Treatment modality and response were comparable in different subtypes of AAV, except for this young MPA group. Additional prospective studies are needed to better understand the different phenotypes of pAAV.

Diagnostic sensitivity and specificity of CT angiography for renal artery stenosis in children.

BACKGROUND: Children with suspected renal artery stenosis (RAS) are screened with renal Doppler ultrasonography or computed tomography (CT) angiography/magnetic resonance (MR) angiography depending on institutional preference. CT angiography produces images with superior resolution, allowing higher quality multiplanar two-dimensional reformats and three-dimensional reconstructions. However, there is a paucity of data in the literature regarding the utility and diagnostic performance of renal CT angiography in pediatric RAS. OBJECTIVE: The objective of this study is to retrospectively review our experience with renal CT angiography in the diagnosis of pediatric RAS relative to digital subtraction angiography (DSA) as the reference standard. MATERIALS AND METHODS: All patients 0-18 years of age who underwent CT angiography for evaluation of RAS as a cause of hypertension between January 2012 and May 2019 were identified for the study. A total of 131 patients were identified, 23 of whom had DSA correlation. RESULTS: Twenty-three patients (17 boys, 6 girls) with a mean age of 6 years 3 months (range: 3 months to 14 years 7 months) were included in this study. Of the 59 renal arteries studied by DSA, 22 were abnormal on CT angiography and 20 were abnormal on DSA. Of the 59 renal arteries, CT angiography was true positive in 18 and true negative in 35. The sensitivity and specificity of CT angiography for RAS diagnosis were 90.0% and 89.7%, respectively. CT angiography identified all cases of main RAS. CONCLUSION: Renal CT angiography has a high sensitivity and specificity for pediatric RAS diagnosis in patients referred for DSA.

Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging.

BACKGROUND: Although spinal magnetic resonance imaging (MRI) findings of neuromyelitis optica (NMO) have been described, there is limited data available that help differentiate NMO from other causes of longitudinally extensive transverse myelitis (LETM). OBJECTIVE: To investigate the spinal MRI findings of LETM that help differentiate NMO at the acute stage from multiple sclerosis (MS) and other causes of LETM. METHODS: We enrolled 94 patients with LETM into our study. Bright spotty lesions (BSL), the lesion distribution and location were evaluated on axial T2-weighted images. Brainstem extension, cord expansion, T1 darkness and lesion enhancement were noted. We also reviewed the brain MRI of the patients during LETM. RESULTS: Patients with NMO had a greater amount of BSL and T1 dark lesions (p < 0.001 and 0.003, respectively). The lesions in NMO patients were more likely to involve greater than one-half of the spinal cord's cross-sectional area; to enhance and be centrally-located, or both centrally- and peripherally-located in the cord. Of the 62 available brain MRIs, 14 of the 27 whom were NMO patients had findings that may be specific to NMO. CONCLUSIONS: Certain spinal cord MRI features are more commonly seen in NMO patients and so obtaining brain MRI during LETM may support diagnosis.

Magnetic Resonance Imaging Perception of the Society in Kars: A Local Study.

BACKGROUND: The start point of this study was the sentence that a patient used: 'my pains had gone with MRI'. It is known that MRI has not a usage area in treatment, yet. Perhaps, the feeling of loss of pain was only a perception. But we want to search the demographic factors that make the perception of loss of pain. The purpose of this study was to determine the consciousness level of the society about MRI. MATERIAL/METHODS: This prospective survey study included 302 (107 men, 195 women; mean age 43.11±15.18 years) patients who were referred to the radiology clinic to undergo MRI. RESULTS: Almost half of the patients were illiterate and graduated from a primary school. Low level of education was more frequent in women than in men. Most of the patients declared that MRI would diagnose their disease. Among all the patients surveyed, 209 of 302 patients indicated no changes in the degree of pain before and after MRI, 30 indicated increased pain, 62 indicated decreased pain, and one patient did not answer the question. Most of the patients who declared decreasing pain had lumbar or cervical MRI. CONCLUSIONS: The function of MRI was known by the patients independently from their educational status. The degree of decrease in pain was higher in the 'treatment' answer. Perhaps the relatively higher percentage was a result of the expectations about treatment and was related with psychological motivation.

Congenital abnormalities of the posterior fossa.

The frequency and importance of the evaluation of the posterior fossa have increased significantly over the past 20 years owing to advances in neuroimaging. Nowadays, conventional and advanced neuroimaging techniques allow detailed evaluation of the complex anatomic structures within the posterior fossa. A wide spectrum of congenital abnormalities has been demonstrated, including malformations (anomalies due to an alteration of the primary developmental program caused by a genetic defect) and disruptions (anomalies due to the breakdown of a structure that had a normal developmental potential). Familiarity with the spectrum of congenital posterior fossa anomalies and their well-defined diagnostic criteria is crucial for optimal therapy, an accurate prognosis, and correct genetic counseling. The authors discuss the spectrum of posterior fossa malformations and disruptions, with emphasis on neuroimaging findings (including diagnostic criteria), neurologic presentation, systemic involvement, prognosis, and risk of recurrence.

Achondroplasia in children: correlation of ventriculomegaly, size of foramen magnum and jugular foramina, and emissary vein enlargement.

PURPOSE: Achondroplasia is a skeletal dysplasia with diminished growth of the skull base secondary to defective enchondral bone formation. This leads to narrowing of the foramen magnum and jugular foramina, which further leads to ventricular dilatation and prominence of the emissary veins. The primary goal of our study was to determine a correlation between the degree of ventricular dilatation, jugular foramina and foramen magnum narrowing, as well as emissary vein enlargement. METHODS: Conventional T2-weighted MR images were evaluated for surface area of the foramen magnum and jugular foramina, ventricular dilatation, and emissary veins enlargement in 16 achondroplasia patients and 16 age-matched controls. Ratios were calculated for the individual parameters using median values from age-matched control groups to avoid age as a confounder. RESULTS: Compared to age-matched controls, in children with achondroplasia, the surface area of the foramen magnum (median 0.50 cm(2), range 0.23-1.37 cm(2) vs. 3.14 cm(2), 1.83-6.68 cm(2), p < 0.001) and jugular foramina (median 0.02 cm(2), range 0-0.10 cm(2) vs. 0.21 cm(2), 0.03-0.61 cm(2), p < 0.001) were smaller, whereas ventricular dilatation (0.28, 0.24-0.4 vs. 0.26, 0.21-0.28, p < 0.001) and enlargement of emissary veins (6, 0-11 vs. 0, p < 0.001) were higher. Amongst the patients, Spearman correlation and multiple regression analysis did not reveal correlation for severity between the individual parameters. CONCLUSIONS: Our study suggests that in children with achondroplasia, (1) the variation in ventricular dilatation may be related to an unquantifiable interdependent relationship of emissary vein enlargement, venous channel narrowing, and foramen magnum compression and (2) stable ventricular size facilitated by interdependent factors likely obviates the need for ventricular shunt placement.

Diffusion tensor imaging of the cervical spinal cord in children.

PURPOSE: Obtaining fast, reliable, high-resolution diffusion tensor imaging (DTI) of the pediatric cervical spinal cord (CSC) is challenging, given the multitude of technical limitations involved. Overcoming these limitations may further potentiate DTI as a valuable quantitative tool in evaluating the pediatric CSC. METHODS: Sixteen patients (9 girls and 7 boys) with hypoxic brain injury, craniocervical junction malformations, and head trauma were included in this retrospective study. Region of interests were placed from C1-C2 through C7-T1 consecutively at the cervical intervertebral disc levels. DTI metrics were compared with a pediatric DTI database of healthy controls. Clinical background and outcomes were tabulated. RESULTS: Patients with hypoxic brain injury, Chiari I and II malformations, and head trauma demonstrated lower fractional anisotropy values than that of healthy controls at certain cervical intervertebral disc levels. CONCLUSIONS: DTI may be a promising modality for providing additional information beyond that of conventional magnetic resonance imaging in pediatric central nervous system disorders.

Diffusion tensor imaging of the brainstem in children with achondroplasia.

AIM: The aims of this study were to compare, using diffusion tensor imaging (DTI) of the brainstem, microstructural integrity of the white matter in children with achondroplasia and age-matched participants and to correlate the severity of craniocervical junction (CCJ) narrowing and neurological findings with DTI scalars in children with achondroplasia. This study also aimed to assess the potential role of fibroblast growth factor receptor type 3 on white matter microstructure. METHOD: Diffusion tensor imaging was performed using a 1.5T magnetic resonance scanner and balanced pairs of diffusion gradients along 20 non-collinear directions. Measurements were obtained from regions of interest, sampled in each pontine corticospinal tract (CST), medial lemniscus, and middle cerebellar peduncle, as well as in the lower brainstem and centrum semiovale, for fractional anisotropy and for mean, axial, and radial diffusivity. In addition, a severity score for achondroplasia was assessed by measuring CCJ narrowing. RESULT: Eight patients with achondroplasia (seven males, one female; mean age 5y 6mo, range 1y 1mo-15y 1mo) and eight age- and sex-matched comparison participants (mean age 5y 2mo, range 1y 1mo-14y 11mo) were included in this study. Fractional anisotropy was lower and mean diffusivity and radial diffusivity were higher in the lower brainstem of patients with achondroplasia than in age-matched comparison participants. The CST and middle cerebellar peduncle of the participants showed increases in mean, axial, and radial diffusivity. Fractional anisotropy in the lower brainstem was negatively correlated with the degree of CCJ narrowing. No differences in the DTI metrics of the centrum semiovale were observed between the two groups. INTERPRETATION: The reduction in fractional anisotropy and increase in diffusivities in the lower brainstem of participants with achondroplasia may reflect secondary encephalomalacic degeneration and cavitation of the affected white matter tracts as shown by histology. In children with achondroplasia, DTI may serve as a potential biomarker for brainstem white matter injury and aid in the care and management of these patients.

Scout view in pediatric CT neuroradiological evaluation: do not underestimate!

PURPOSE: Computed tomography (CT) is an important first-line imaging tool in pediatric neuroradiology. The scout view (SV) is essential for planning the CT study. It is frequently underestimated for its diagnostic value but may harbor important diagnostic clues. We present and discuss the role of SV in the accurate interpretation of pediatric neuroradiological CT studies. METHODS: CT studies have been collected over 12 months by an experienced pediatric neuroradiologist. Retrospective evaluation of SV and axial CT images was performed in nine children, where the SV gave important diagnostic information. Abnormalities on SV were classified as (1) located outside the field of view as imaged by the cross-sectional CT slices or (2) located in the plane of the cross-sectional CT slices or orthogonal to it. RESULTS: Five male and four female patients were included. The mean age at CT was 9.86 years (range, 0.75 to 19 years). Abnormalities on SV were located outside of the field of view as imaged by cross-sectional CT slices in six out of nine children and were within the plane of the CT slices or orthogonal to it in three out of nine children. CONCLUSIONS: A thorough and accurate evaluation of the SV may add valuable, clinically relevant information and should be a routine part of the interpretation of each pediatric neuroradiological CT study.

Presentation of External Ear Rosai-Dorfman Disease With Laryngeal Involvement.

Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered to be benign, death can occur depending on the extent and location. Our case highlights a primary extranodal site of the right pinna with extension through the Eustachian tube to the subglottis. A previously healthy 15-year-old female presented with 1-year right pinna swelling, slowly enlarging and becoming more bothersome. An incisional biopsy was performed on the ear along with S100 staining yielding a diagnosis. After multidisciplinary case discussion, clofarabine monotherapy and systemic therapy for Langerhans cell histiocytosis has started. Rosai-Dorfman disease can be a general disorder, often affecting the lymph nodes. Unlike a nodal disease, extranodal disease could involve any site on the patient's anatomy. Head and neck lesions are the most common extranodal lesions. Rosai-Dorfman disease is self-limited in more than 20% of the cases with spontaneous regression without intervention; 70% of the patients have noticeable symptoms and vital organ involvement requiring treatments such as surgery, steroids, radiation, and chemotherapy. In our case, the patient had wide involvement and presented without any serious breathing difficulties; we decided to start with monotherapy with chemotherapy and systematic glucocorticoid treatment.

Persistent left superior vena cava draining to the left atrium: A case report and review of the literature.

BACKGROUND: Persistent left superior vena cava is a rare but important congenital vascular anomaly. However, PLSVC with absent RSVC (isolated PLSVC) is a very rare venous malformation We report on a rare case of persistent left superior vena cava (PLSVC) with absent right superior vena cava (RSVC). CASE REPORT: This venous malformation was identified incidentally in a 69-year-old woman during chest multi-detector computed tomography (MDCT). On chest MDCT, the SVC was noted on the left side. A bridging vein drained the right jugular and right subclavian veins and joined the left brachiocephalic vein in order to form the PLSVC, which descended on the left side of the mediastinum and drained into the left atrium (LA). The patient had no additional cardiac anomaly. CONCLUSIONS: Isolated PLSVC is usually asymptomatic but it can pose difficulties for establishing central venous access, pacemaker implantation and cardiothoracic surgery. This condition is also associated with an increased incidence of congenital heart disease, arrhythmias and conduction disturbances. A wide spectrum of clinicians should be aware of this anomaly, its variations and possible complications.

Neuroimaging of primary mitochondrial disorders in children: A review.

Mitochondrial disorders represent a diverse and complex group of entities typified by defective energy metabolism. The mitochondrial oxidative phosphorylation system is typically impaired, which is the predominant source of energy production. Because mitochondria are present in nearly all organs, multiple systems may be affected including the central nervous system, skeletal muscles, kidneys, and liver. In particular, those organs that are metabolically active with high energy demands are explicitly vulnerable. Initial diagnostic work up relies on a detailed evaluation of clinical symptoms including physical examination as well as a comprehensive review of the evolution of symptoms over time, relation to possible "triggering" events (eg, fever, infection), blood workup, and family history. High-end neuroimaging plays a pivotal role in establishing diagnosis, narrowing differential diagnosis, monitoring disease progression, and predicting prognosis. The pattern and characteristics of the neuroimaging findings are often highly suggestive of a mitochondrial disorder; unfortunately, in many cases the wide variability of involved metabolic processes prevents a more specific subclassification. Consequently, additional diagnostic steps including muscle biopsy, metabolic workup, and genetic tests are necessary. In the current manuscript, basic concepts of energy production, genetics, and inheritance patterns are reviewed. In addition, the imaging findings of several illustrative mitochondrial disorders are presented to familiarize the involved physicians with pediatric mitochondrial disorders. In addition, the significance of spinal cord imaging and the value of "reversed image-based discovery" for the recognition and correct (re-)classification of mitochondrial disorders is discussed.

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.

Neuroimaging in cerebellar ataxia in childhood: A review.

Ataxia is one of the most common pediatric movement disorders and can be caused by a large number of congenital and acquired diseases affecting the cerebellum or the vestibular or sensory system. It is mainly characterized by gait abnormalities, dysmetria, intention tremor, dysdiadochokinesia, dysarthria, and nystagmus. In young children, ataxia may manifest as the inability or refusal to walk. The diagnostic approach begins with a careful clinical history including the temporal evolution of ataxia and the inquiry of additional symptoms, is followed by a meticulous physical examination, and, depending on the results, is complemented by laboratory assays, electroencephalography, nerve conduction velocity, lumbar puncture, toxicology screening, genetic testing, and neuroimaging. Neuroimaging plays a pivotal role in either providing the final diagnosis, narrowing the differential diagnosis, or planning targeted further workup. In this review, we will focus on the most common form of ataxia in childhood, cerebellar ataxia (CA). We will discuss and summarize the neuroimaging findings of either the most common or the most important causes of CA in childhood or present causes of pediatric CA with pathognomonic findings on MRI. The various pediatric CAs will be categorized and presented according to (a) the cause of ataxia (acquired/disruptive vs. inherited/genetic) and (b) the temporal evolution of symptoms (acute/subacute, chronic, progressive, nonprogressive, and recurrent). In addition, several illustrative cases with their key imaging findings will be presented.

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.