A Hemorrhagic Brain Mass in a Child With Encephalocraniocutaneous Lipomatosis.

Encephalocraniocutaneous lipomatosis (ECCL) is a rare genetic condition with well-described skin, ocular, and central nervous system findings. Several case reports have been documented demonstrating the presence of low-grade gliomas in patients with ECCL and the association with certain FGFR1 mutations. We report on a case of diffuse low-grade glioma, mitogen activated protein kinase pathway altered in a patient with ECCL, who was found to have a distinct FGFR1 mutation.

Near-pair patch generative adversarial network for data augmentation of focal pathology object detection models.

Purpose: The limited volume of medical training data remains one of the leading challenges for machine learning for diagnostic applications. Object detectors that identify and localize pathologies require training with a large volume of labeled images, which are often expensive and time-consuming to curate. To reduce this challenge, we present a method to support distant supervision of object detectors through generation of synthetic pathology-present labeled images. Approach: Our method employs the previously proposed cyclic generative adversarial network (cycleGAN) with two key innovations: (1) use of "near-pair" pathology-present regions and pathology-absent regions from similar locations in the same subject for training and (2) the addition of a realism metric (Fréchet inception distance) to the generator loss term. We trained and tested this method with 2800 fracture-present and 2800 fracture-absent image patches from 704 unique pediatric chest radiographs. The trained model was then used to generate synthetic pathology-present images with exact knowledge of location (labels) of the pathology. These synthetic images provided an augmented training set for an object detector. Results: In an observer study, four pediatric radiologists used a five-point Likert scale indicating the likelihood of a real fracture (1 = definitely not a fracture and 5 = definitely a fracture) to grade a set of real fracture-absent, real fracture-present, and synthetic fracture-present images. The real fracture-absent images scored 1.7±1.0, real fracture-present images 4.1±1.2, and synthetic fracture-present images 2.5±1.2. An object detector model (YOLOv5) trained on a mix of 500 real and 500 synthetic radiographs performed with a recall of 0.57±0.05 and an F2 score of 0.59±0.05. In comparison, when trained on only 500 real radiographs, the recall and F2 score were 0.49±0.06 and 0.53±0.06, respectively. Conclusions: Our proposed method generates visually realistic pathology and that provided improved object detector performance for the task of rib fracture detection.

Surface Reconstruction of the Pediatric Larynx via Structure from Motion Photogrammetry: A Pilot Study.

Endoscopy is the gold standard for characterizing pediatric airway disorders, however, it is limited for quantitative analysis due to lack of three-dimensional (3D) vision and poor stereotactic depth perception. We utilize structure from motion (SfM) photogrammetry, to reconstruct 3D surfaces of pathologic and healthy pediatric larynges from monocular two-dimensional (2D) endoscopy. Models of pediatric subglottic stenosis were 3D printed and airway endoscopies were simulated. 3D surfaces were successfully reconstructed from endoscopic videos of all models using an SfM analysis toolkit. Average subglottic surface error between SfM reconstructed surfaces and 3D printed models was 0.65 mm as measured by Modified Hausdorff Distance. Average volumetric similarity between SfM surfaces and printed models was 0.82 as measured by Jaccard Index. SfM can be used to accurately reconstruct 3D surface renderings of the larynx from 2D endoscopy video. This technique has immense potential for use in quantitative analysis of airway geometry and virtual surgical planning.

Maternal COVID-19 Infection Associated with Fetal Systemic Inflammatory Complications in COVID-19-Negative Neonates: A Case-Series.

OBJECTIVE: This study aimed to examine fetal and neonatal inflammatory and neurologic complications associated with maternal coronavirus disease 2019 (COVID-19) infection. STUDY DESIGN: Case-series using a convenience sample of neonates cared for in a large referral-based children's hospital neonatal intensive care unit between September 2021 and May 2022. RESULTS: We identified seven neonates with exposure to maternal severe acute respiratory syndrome related coronavirus 2 (SARS-CoV-2) and a presentation consistent with inflammatory complications. All had some degree of neurologic injury with neuroimaging findings including restricted diffusion indicating injury in the white matter, cortex, deep gray structures, and splenium of the corpus callosum as well as intracranial hemorrhage. In addition, many infants had cytopenia and abnormal coagulation studies. Placental pathology, when available, revealed inflammation, clot with calcifications, and hematomas with associated infarcts. CONCLUSION: Neonates born to mothers with SARS-CoV-2, even when negative for the virus themselves, may have complications consistent with a systemic inflammatory syndrome. Placental pathology as well as neurologic imaging in infants with neurologic findings may help to support this diagnosis. KEY POINTS: · A systemic inflammatory response may cause illness in babies born to mothers with a history of COVID-19.. · Inflammatory markers and placental pathology are helpful in supporting this diagnosis.. · Consider neuroimaging in infants of mothers with a history of COVID-19 with neurologic findings..

Changes to pediatric brain tumors in 2021 World Health Organization classification of tumors of the central nervous system.

New tumor types are continuously being described with advances in molecular testing and genomic analysis resulting in better prognostics, new targeted therapy options and improved patient outcomes. As a result of these advances, pathological classification of tumors is periodically updated with new editions of the World Health Organization (WHO) Classification of Tumors books. In 2021, WHO Classification of Tumors of the Central Nervous System, 5th edition (CNS5), was published with major changes in pediatric brain tumors officially recognized including pediatric gliomas being separated from adult gliomas, ependymomas being categorized based on anatomical compartment and many new tumor types, most of them seen in children. Additional general changes, such as tumor grading now being done within tumor types rather than across entities and changes in definition of glioblastoma, are also relevant to pediatric neuro-oncology practice. The purpose of this manuscript is to highlight the major changes in pediatric brain tumors in CNS5 most relevant to radiologists. Additionally, brief descriptions of newly recognized entities will be presented with a focus on imaging findings.

Intraventricular B7-H3 CAR T Cells for Diffuse Intrinsic Pontine Glioma: Preliminary First-in-Human Bioactivity and Safety.

Diffuse intrinsic pontine glioma (DIPG) remains a fatal brainstem tumor demanding innovative therapies. As B7-H3 (CD276) is expressed on central nervous system (CNS) tumors, we designed B7-H3-specific chimeric antigen receptor (CAR) T cells, confirmed their preclinical efficacy, and opened BrainChild-03 (NCT04185038), a first-in-human phase I trial administering repeated locoregional B7-H3 CAR T cells to children with recurrent/refractory CNS tumors and DIPG. Here, we report the results of the first three evaluable patients with DIPG (including two who enrolled after progression), who received 40 infusions with no dose-limiting toxicities. One patient had sustained clinical and radiographic improvement through 12 months on study. Patients exhibited correlative evidence of local immune activation and persistent cerebrospinal fluid (CSF) B7-H3 CAR T cells. Targeted mass spectrometry of CSF biospecimens revealed modulation of B7-H3 and critical immune analytes (CD14, CD163, CSF-1, CXCL13, and VCAM-1). Our data suggest the feasibility of repeated intracranial B7-H3 CAR T-cell dosing and that intracranial delivery may induce local immune activation. SIGNIFICANCE: This is the first report of repeatedly dosed intracranial B7-H3 CAR T cells for patients with DIPG and includes preliminary tolerability, the detection of CAR T cells in the CSF, CSF cytokine elevations supporting locoregional immune activation, and the feasibility of serial mass spectrometry from both serum and CSF. This article is highlighted in the In This Issue feature, p. 1.

Association Between Early EEG Background and Outcomes in Infants With Mild HIE Undergoing Therapeutic Hypothermia.

BACKGROUND: Hypoxic-ischemic encephalopathy (HIE) occurs in 1-4:1000 live births. Although neonates with moderate-severe HIE have been studied over several decades, newborns with mild HIE remain understudied, including seizure occurrence, electroencephalography (EEG) characteristics, and outcome. METHODS: We conducted a retrospective cohort study of neonates ≥35 weeks of gestation with mild HIE who underwent therapeutic hypothermia to correlate the early EEG background pattern with clinical course and outcomes. RESULTS: Of the included 29 neonates, 10 infants had a moderately to severely abnormal EEG background and 19 had either a normal or a mildly abnormal background. Those with moderately to severely abnormal background also had more multiorgan dysfunction (90% vs 42%, P = 0.02) and a higher incidence of subdural and intraventricular hemorrhages (80% vs 26%, P = 0.02). The overall seizure incidence was 20.7% and was significantly higher in newborns with more severely abnormal background compared to neonates with less abnormal background (50% vs 5%; P = 0.01; relative risk, 9.5; 95% confidence interval, 1.28-70.6). Seizure onset was between 11 and 63 hours of life. Regardless of the EEG background pattern, seizures were brief with an overall low seizure burden. None of the newborns with normal or mildly abnormal background had a new onset of seizures after 24 hours of recording or developed epilepsy during infancy. CONCLUSIONS: In neonates with mild HIE, early moderately to severely abnormal EEG background is common and strongly associated with an increased risk for seizures.

Mitochondria-Associated Membrane Scaffolding with Endoplasmic Reticulum: A Dynamic Pathway of Developmental Disease.

Communication between intracellular organelles is essential for overall cellular function. How this communication occurs and under what circumstances alterations transpire are only the beginning to be elucidated. The pathways of calcium homeostasis, lipid transfer, mitochondrial dynamics, and mitophagy/apoptosis have been linked to the endoplasmic reticulum and tethering sites on the outer and/or inner mitochondrial membrane called mitochondria-associated endoplasmic reticulum membranes (MAM). Sensitive visualization by high-powered microscopy coupled with the advent of massive parallel sequencing has elaborated the structure, while patient's diseases have uncovered the physiological function of these networks. Using specific patient examples from our pediatric mitochondrial center, we expand how specific genetic pathological variants in certain MAM structures induce disease. Genetic variants in MICU1, PASC-2, CYP2U1, SERAC1, and TANGO2 can induce early development abnormalities in the areas of cognition, motor, and central nervous system structures across multiple MAM pathways and implicate mitochondrial dysregulation.

Auriculocondylar syndrome 2 results from the dominant-negative action of PLCB4 variants.

Auriculocondylar syndrome 2 (ARCND2) is a rare autosomal dominant craniofacial malformation syndrome linked to multiple genetic variants in the coding sequence of phospholipase C ß4 (PLCB4). PLCB4 is a direct signaling effector of the endothelin receptor type A (EDNRA)-Gq/11 pathway, which establishes the identity of neural crest cells (NCCs) that form lower jaw and middle ear structures. However, the functional consequences of PLCB4 variants on EDNRA signaling is not known. Here, we show, using multiple signaling reporter assays, that known PLCB4 variants resulting from missense mutations exert a dominant-negative interference over EDNRA signaling. In addition, using CRISPR/Cas9, we find that F0 mouse embryos modeling one PLCB4 variant have facial defects recapitulating those observed in hypomorphic Ednra mouse models, including a bone that we identify as an atavistic change in the posterior palate/oral cavity. Remarkably, we have identified a similar osseous phenotype in a child with ARCND2. Our results identify the disease mechanism of ARCND2, demonstrate that the PLCB4 variants cause craniofacial differences and illustrate how minor changes in signaling within NCCs may have driven evolutionary changes in jaw structure and function. This article has an associated First Person interview with the first author of the paper.

Extra-axial haemorrhages in young children with skull fractures: abuse or accident?

OBJECTIVE: Infant and toddler subdural haemorrhages (SDH) are often considered indicative of abuse or major trauma. However, accidental impact events, such as falls, cause contact extra-axial haemorrhages (EAHs). The current study sought to determine frequency and clinical behaviour of EAHs with infant and toddler accidental and abusive skull fractures. PATIENTS AND METHODS: Children aged <4 years with accidental skull fractures and abusive fractures identified by CT at two paediatric tertiary care centres. Clinical data were abstracted by child abuse paediatricians and images were reviewed by paediatric radiologists. Data were analysed using univariate and multivariate logistic regression as well as descriptive statistics. RESULTS: Among 227 subjects, 86 (37.9%) had EAHs. EAH was present in 73 (34.8%) accidental and 13 (76.5%) of the abusive injuries. Intracranial haemorrhage rates were not different for children with major or minor accidents but were fewer than abused. EAH was equally common with falls <4 and >4 ft. EAH depths did not differ by mechanism, but 69% of accidental EAHs were localised solely at fracture sites vs 38% abuse. Widespread and multifocal EAHs were more common with abuse. Children with abuse or major accidental injuries presented with lower initial Glasgow Coma Scales than those with minor accidents. Abused children had initial loss of consciousness more often than those with either minor or major accidents. CONCLUSIONS: Simple contact EAHs were common among children with minor and major accidental skull fractures. Accidental EAHs were more localised with less neurological dysfunction than abusive.

Normative Values for the Sonographic Measurement of the Pediatric Median and Ulnar Nerves.

RATIONAL AND OBJECTIVES: Ultrasound investigation of peripheral nerves, long used in the adult population, has been shown to be of value in diagnosing a variety of peripheral nerve abnormalities. More recently, nerve sonography has also been shown to be of use in pediatrics. However, normative values for nerve size in children have been lacking. As such, the goal of this research was to establish normative data for cross sectional area (CSA) measurements of the median and ulnar nerves in children. MATERIALS AND METHODS: The median and ulnar nerves of 48 children ranging in age from 2 years to 17 years were imaged by ultrasound. CSA measurements were made at 2 separate sites for each nerve and measured independently by 6 pediatric radiologists. Reliability of ultrasound measurements between the radiologists was assessed by calculating intraclass correlation coefficients. Linear mixed-effects modeling was performed to develop prediction models for nerve cross sectional area for each nerve segment; 95% prediction values were generated from these models to approximate normal ranges. RESULTS: Agreement in nerve cross-sectional area measurements among the 6 radiologists for all nerve segments was good (ICC 0.82, 95% CI 0.78 to 0.85). CSA for both the median and ulnar nerves is larger in older children. However, statistical models to predict CSA using height perform better rather than those using a child's age. The range of normal nerve segment CSA using these prediction models based on child height are reported. CONCLUSION: Median and ulnar nerve CSA can be reliably measured with ultrasound. Normal reference values for ulnar and median nerve CSA correlate with patient age but may be more optimally determined based on a child's height.

Are Complex Skull Fractures Indicative of Either Child Abuse or Major Trauma in the Era of 3-Dimensional Computed Tomography Imaging?

OBJECTIVE: The aim of the study was to determine whether complex skull fractures are more indicative of child abuse or major trauma than simple skull fractures. DESIGN: This is a retrospective chart and imaging review of children diagnosed with a skull fracture. Subjects were from 2 pediatric tertiary care centers. Children younger than 4 years who underwent a head computed tomography with 3-dimensional rendering were included. We reviewed the medical records and imaging for type of skull fracture, abuse findings, and reported mechanism of injury. A complex skull fracture was defined as multiple fractures of a single skull bone, fractures of more than 1 skull bone, a nonlinear fracture, or diastasis of greater than 3 mm. Abuse versus accident was determined at the time of the initial evaluation with child abuse physician team confirmation. RESULTS: From 2011 to 2012, 287 subjects were identified by International Classification of Diseases, Ninth Revision, code. The 147 subjects with a cranial vault fracture and available 3-dimensional computed tomography composed this study's subjects. The average age was 12.3 months. Seventy four (50.3%) had complex and 73 (49.7%) had simple fractures. Abuse was determined in 6 subjects (4.1%), and a determination could not be made for 5 subjects. Adding abused children from 2013 to 2014 yielded 15 abused subjects. Twelve of the abused children (80%) had complex fractures; more than the 66 (48.5%) of 136 accidentally injured children (P = 0.001; relative risk = 1.65 [1.21-2.24]). However, among children with a complex fracture, the positive predictive value for abuse was only 7%. CONCLUSIONS: Complex skull fractures frequently occur from accidental injuries. This study suggests that the presence of complex skull fractures should not be used alone when making a determination of abuse.

Non-inferiority of a non-gadolinium-enhanced magnetic resonance imaging follow-up protocol for isolated optic pathway gliomas.

BACKGROUND: Pediatric patients with optic pathway gliomas (OPGs) typically undergo a large number of follow-up MRI brain exams with gadolinium-based contrast media (GBCM), which have been associated with gadolinium tissue retention. Therefore, careful consideration of GBCM use in these children is warranted. OBJECTIVE: To investigate whether GBCM is necessary for OPG MR imaging response assessment using a blinded, non-inferiority, multi-reader study. MATERIALS AND METHODS: We identified children with OPG and either stable disease or change in tumor size on MRI using a regional cancer registry serving the U.S. Pacific Northwest. For each child, the two relevant, consecutive MRI studies were anonymized and standardized into two imaging sets excluding or including GBCM-enhanced images. Exam pairs were compiled from 42 children with isolated OPG (19 with neurofibromatosis type 1), from a population of 106 children with OPG. We included 28 exam pairs in which there was a change in size between exams. Seven pediatric radiologists measured tumor sizes during three blinded sessions, spaced by at least 1 week. The first measuring session excluded GBCM-enhanced sequences; the others did not. The primary endpoint was intra-reader agreement for ≥ 25% change in axial cross-product measurement, using a 12% non-inferiority threshold. RESULTS: Analysis demonstrated an overall 1.2% difference (95% confidence interval, -3.2% to 5.5%) for intra-reader agreement using a non-GBCM-enhanced protocol and background variability. CONCLUSION: A non-GBCM-enhanced protocol was non-inferior to a GBCM-enhanced protocol for assessing change in size of isolated OPGs on follow-up MRI exams.

The current and future roles of artificial intelligence in pediatric radiology.

Artificial intelligence (AI) is a broad and complicated concept that has begun to affect many areas of medicine, perhaps none so much as radiology. While pediatric radiology has been less affected than other radiology subspecialties, there are some well-developed and some nascent applications within the field. This review focuses on the use of AI within pediatric radiology for image interpretation, with descriptive summaries of the literature to date. We highlight common features that enable successful application of the technology, along with some of the limitations that can inhibit the development of this field. We present some ideas for further research in this area and challenges that must be overcome, with an understanding that technology often advances in unpredictable ways.

Improving the detection of ventricular shunt disruption using volume-rendered three-dimensional head computed tomography.

Hydrocephalus is the most common neurosurgical disorder in children, and cerebrospinal fluid (CSF) diversion with shunt placement is the most commonly performed pediatric neurosurgical procedure. CT is frequently used to evaluate children with suspected CSF shunt malfunction to assess change in ventricular size. Moreover, careful review of the CT images is important to confirm the integrity of the imaged portions of the shunt system. Subtle shunt disruptions can be missed on multiplanar two-dimensional (2-D) CT images, especially when the disruption lies in the plane of imaging. The use of volume-rendered CT images enables radiologists to view the extracranial shunt tubing within the field of view as a three-dimensional (3-D) object. This allows for a rapid and intuitive method of assessing the integrity of the extracranial shunt tubing. The purpose of this pictorial essay is to discuss how volume-rendered CT images can be generated to evaluate CSF shunts in the pediatric population and to provide several examples of their utility in diagnosing shunt disruption. We also address the potential pitfalls of this technique and ways to avoid them.

Accuracy and Reliability of 4D-CT and Flexible Laryngoscopy in Upper Airway Evaluation in Robin Sequence.

OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiber-optic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day time points, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 32 sets of 4D-CT visualizations and FFL videos (dynamic modalities) and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1 = none to 5 = complete) and noted confidence levels of each rating. Intraclass correlation and Krippendorff alpha were used to assess intra- and interrater reliability, respectively. Accuracy was assessed by comparing clinician ratings with quantitative percentage constriction (QPC) ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: There was similar intrarater agreement (moderate to substantial) with 4D-CT and FFL, and both demonstrated fair interrater agreement. Both modalities underestimated UAO severity, although 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (-1.06 and -1.46 vs QPC ratings, P = .004). Overall confidence levels were similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, P < .0001). CONCLUSION: Although 4D-CT may be more accurate in assessing the degree of UAO in patients with RS, 4D-CT and FFL assessments demonstrate similar reliability. Additionally, 4D-CT may be interpreted with greater confidence by nonotolaryngologists who care for these patients.

Evaluating the Utility of Routine Computed Tomography Scans after Cranial Vault Reconstruction for Children with Craniosynostosis.

BACKGROUND: Postoperative computed tomography scans allow for evaluation of the structural results of cranial vault reconstruction and potential surgical concerns. The authors evaluated the clinical utility of routine postoperative scans to identify relevant surgical findings in children treated for craniosynostosis. METHODS: The authors conducted a retrospective study of postoperative computed tomography reports for patients with craniosynostosis following cranial vault reconstruction during a 9-year period at their tertiary care pediatric hospital. They categorized postoperative computed tomography findings as typical, atypical, or indeterminate. Images with reported indeterminate or atypical findings were reviewed and verified by a pediatric neuroradiologist and a pediatric neurological surgeon. Clinical outcomes of patients with abnormal postoperative images were assessed with chart review for clinical relevance. RESULTS: Postoperative computed tomography radiology reports for 548 operations in 506 participants were included. Most participants had single-suture craniosynostosis (89 percent), were male (64 percent), and under 1 year of age (78 percent). Surgically concerning scans were described in 52 reports (<9.5 percent), and the research team's pediatric neuroradiologist confirmed abnormal findings in 36 (6.5 percent). Potentially relevant abnormal findings included subdural blood (n = 18), subarachnoid blood (n = 4), intraparenchymal findings (n = 6), bone abnormalities (n = 5), vascular injury (n = 3), and increased ventricular size (n = 2). Most cases with abnormal findings did not require additional observation nor intervention. Only three cases (of 548; 0.55 percent) required further intervention, which included additional medical management (n = 2) and return to the operating room (n = 1). CONCLUSION: Abnormal findings on routine computed tomography scans after cranial vault reconstruction are uncommon and rarely result in an urgent surgical or medical intervention. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, IV.

Automated atlas-based segmentation for skull base surgical planning.

PURPOSE: Computational surgical planning tools could help develop novel skull base surgical approaches that improve safety and patient outcomes. This defines a need for automated skull base segmentation to improve the usability of surgical planning software. The objective of this work was to design and validate an algorithm for atlas-based automated segmentation of skull base structures in individual image sets for skull base surgical planning. METHODS: Advanced Normalization Tools software was used to construct a synthetic CT template from 6 subjects, and skull base structures were manually segmented to create a reference atlas. Landmark registration followed by Elastix deformable registration was applied to the template to register it to each of the 30 trusted reference image sets. Dice coefficient, average Hausdorff distance, and clinical usability scoring were used to compare the atlas segmentations to those of the trusted reference image sets. RESULTS: The mean for average Hausdorff distance for all structures was less than 2 mm (mean for 95th percentile Hausdorff distance was less than 5 mm). For structures greater than 2.5 mL in volume, the average Dice coefficient was 0.73 (range 0.59-0.82), and for structures less than 2.5 mL in volume the Dice coefficient was less than 0.7. The usability scoring survey was completed by three experts, and all structures met the criteria for acceptable effort except for the foramen spinosum, rotundum, and carotid artery, which required more than minor corrections. CONCLUSION: Currently available open-source algorithms, such as the Elastix deformable algorithm, can be used for automated atlas-based segmentation of skull base structures with acceptable clinical accuracy and minimal corrections with the use of the proposed atlas. The first publicly available CT template and anterior skull base segmentation atlas being released (available at this link: http://hdl.handle.net/1773/46259 ) with this paper will allow for general use of automated atlas-based segmentation of the skull base.

Focal Cerebral Arteriopathy of Childhood: Clinical and Imaging Correlates.

Background and Purpose: Focal cerebral arteriopathy (FCA) of childhood with unilateral stenosis of the anterior circulation is reported to account for up to one-quarter of childhood arterial ischemic stroke, with stroke recurrence in 25% of cases. Limited knowledge regarding pathophysiology and outcome results in inconsistent treatment of FCA. Methods: Children with arterial ischemic stroke due to FCA between January 1, 2009, and January 1, 2019, were retrospectively identified at our institution which serves the US Pacific Northwest region. Electronic health record data, including neuroimaging studies, were reviewed, and the Pediatric Stroke Outcome Measure at 1 year was determined as the primary clinical end point. Results: Fifteen children were diagnosed with FCA, accounting for 19% of children with cerebral arteriopathies (n=77). Among children with FCA, the median age at the time of stroke was 6.8 years (Q1­Q3, 1.9­14.0 years). Four (20%) patients had worsening stroke, 3 of whom had concurrent infection. Three (20%) FCA cases were treated with steroids, one of whom had worsening stroke. Median Pediatric Stroke Outcome Measure at 1 year was 1.0 (Q1­Q3, 0.6­2.0). Variability in arteriopathy severity was observed within many patients. Patients with more severe arteriopathy using the Focal Cerebral Arteriopathy Severity Score had larger strokes and were more likely to have worsening stroke. The most common long-term neurological deficit was hemiparesis, which was present in 11 (73%) patients and associated with middle cerebral artery arteriopathy and infarcts. Conclusions: FCA may be less common than previously reported. Neuroimaging in FCA can help identify patients at greater risk for worsening stroke.