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Purpose: Brain arteriovenous malformations (bAVM) are the main cause of pediatric intracerebral hemorrhage (pICH). Embolization with Onyx (ev3, Irvine, CA, USA) in children with ruptured bAVM has been infrequently reported. The aim of this study was to assess the safety and efficacy profile of Onyx embolization as first line endovascular treatment of ruptured pediatric bAVMs., Methods: Children with non-traumatic pICH due to bAVM rupture at a pediatric quaternary care center were prospectively enrolled in a registry and retrospectively analyzed between 2013 and 2018. Clinical and demographic data, treatment modalities and clinical imaging follow-up were retrieved, and detailed procedural data were retrospectively assessed by two investigators. The safety (procedural morbidity and mortality) and efficacy (obliteration and interval rebleeding) were evaluated., Results: In this study 29 children treated for a bAVM by Onyx embolization were included (14 girls, 48%; median age 11.1 years, interquartile range, IQR 8.1-12.7 years) with a total of 72 endovascular sessions (median of 2 sessions per patient IQR 1-3). The AVMs were deeply located in 23 patients (79%). No systemic complications occurred, and no child experienced embolization-related persistent neurological deficits. Non-clinically relevant complications were observed during five procedures, unrelated to Onyx. After a mean follow-up of 31 months from rupture complete obliteration rates were 100%, 89%, 29%, 14% in bAVM Spetzler Martin grades I, II, III and IV-V, respectively., Conclusion: It was found that Onyx embolization is safe and represents a good option for an initial treatment approach, in a sequential treatment strategy for pediatric ruptured brain AVMs. Younger age may not be an argument to deny Onyx embolization.

Background and Purpose: Significant hemorrhage expansion (sHE) is a known predictor of poor outcome after an intracerebral hemorrhage (ICH) in adults but remains poorly reported in children. In a large inception cohort, we aimed to explore the prevalence of sHE, its associations with clinical outcomes, and its clinical-imaging predictors in children., Methods: Children admitted between January 2000 and March 2020 at a quaternary care pediatric hospital were screened for inclusion. Sample was restricted to children with 2 computed tomography scans within 72 hours of ICH onset, and a minimal clinical follow-up of months. sHE was defined as an increase from baseline ICH volume by 6 cc or 33% on follow-up computed tomography. Clinical outcome was assessed at 12 months with the King's Outcome Scale for Childhood Head Injury score and defined as favorable for scores ≥5., Results: Fifty-two children met inclusion criteria, among which 8 (15%) demonstrated sHE, and 18 (34.6%) any degree of expansion. Children with sHE had more frequent coagulation disorders (25.0% versus 2.3%; P =0.022). After multivariable adjustment, only the presence of coagulation disorders at baseline remained independently associated with sHE (adjusted odds ratio, 14.4 [95% CI, 1.04-217]; P =0.048). sHE was independently associated with poor outcome (King's Outcome Scale for Childhood Head Injury <5A, odds ratio, 5.77 [95% CI, 1.01-38.95]; P =0.043)., Conclusions: sHE is a frequent phenomenon after admission for a pediatric ICH and more so in children with coagulation defects. As sHE was strongly associated with poorer clinical outcomes, these data mandate a baseline coagulation work up and questions the need for protocolized repeat head computed tomography in children admitted for pediatric ICH.

Background: Gorham-Stout disease (GSD) is a rare lymphatic disorder which results in bone destruction. Defects of the skull base are difficult to manage, we describe cases to better understand the disease and discuss treatment., Methods: Retrospective study including all patients treated for GSD skull-base defects. Medical records, clinical, imaging and treatment data were studied. A systematic review of the literature included case reports of the diseases for further analysis., Results: 6 patients (5 males, 1 female) were included. Mean age at diagnosis was 3.5 years (range 0-10). Follow-up was of 5.2 years. Patients were divided into Naso-temporal (NT) and Vertebro-temporal (VT) groups following anatomical location. NT patients (4 patients) all had petrous defects extending anteriorly, including sphenoid, ethmoidal and mandibular defects. They all had cerebro-spinal fluid leak (CSF) and recurrent meningitis (range from 3 to 7). Two of those patients had sequelae including deafness, paralysis and epilepsy. VT patients (2 patients) all had temporal, occipital bone and cervical vertebrae defects. None had CSF leaks but both died from medullar compression (preceded by tetraparesis in one case). Overall, five out of six patients had type I Chiari malformation. Interferon seemed to be the most efficient medical treatment. Surgery included petrectomy, endonasal surgery for CSF leak management and neurosurgery for medullar management but could not guarantee long-term effects., Conclusion: Main issues in skull base defects are CSF leaks and medullar compressions. Surgical treatment is necessary in both cases but can only be satisfactory if general medical treatment can stabilise the disease., (Copyright © 2019 Elsevier B.V. All rights reserved.)

In patients with pharmaco-resistant focal epilepsies investigated with intracranial electroencephalography (iEEG), direct electrical stimulations of a cortical region induce cortico-cortical evoked potentials (CCEP) in distant cerebral cortex, which properties can be used to infer large scale brain connectivity. In 2013, we proposed a new probabilistic functional tractography methodology to study human brain connectivity. We have now been revisiting this method in the F-TRACT project (f-tract.eu) by developing a large multicenter CCEP database of several thousand stimulation runs performed in several hundred patients, and associated processing tools to create a probabilistic atlas of human cortico-cortical connections. Here, we wish to present a snapshot of the methods and data of F-TRACT using a pool of 213 epilepsy patients, all studied by stereo-encephalography with intracerebral depth electrodes. The CCEPs were processed using an automated pipeline with the following consecutive steps: detection of each stimulation run from stimulation artifacts in raw intracranial EEG (iEEG) files, bad channels detection with a machine learning approach, model-based stimulation artifact correction, robust averaging over stimulation pulses. Effective connectivity between the stimulated and recording areas is then inferred from the properties of the first CCEP component, i.e. onset and peak latency, amplitude, duration and integral of the significant part. Finally, group statistics of CCEP features are implemented for each brain parcel explored by iEEG electrodes. The localization (coordinates, white/gray matter relative positioning) of electrode contacts were obtained from imaging data (anatomical MRI or CT scans before and after electrodes implantation). The iEEG contacts were repositioned in different brain parcellations from the segmentation of patients' anatomical MRI or from templates in the MNI coordinate system. The F-TRACT database using the first pool of 213 patients provided connectivity probability values for 95% of possible intrahemispheric and 56% of interhemispheric connections and CCEP features for 78% of intrahemisheric and 14% of interhemispheric connections. In this report, we show some examples of anatomo-functional connectivity matrices, and associated directional maps. We also indicate how CCEP features, especially latencies, are related to spatial distances, and allow estimating the velocity distribution of neuronal signals at a large scale. Finally, we describe the impact on the estimated connectivity of the stimulation charge and of the contact localization according to the white or gray matter. The most relevant maps for the scientific community are available for download on f-tract. eu (David et al., 2017) and will be regularly updated during the following months with the addition of more data in the F-TRACT database. This will provide an unprecedented knowledge on the dynamical properties of large fiber tracts in human., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Focal cortical dysplasia (FCD) is the most common cause of intractable focal epilepsy in children, in whom seizures are most commonly pharmacoresistant from onset. This review summarizes the current understandings of the epidemiology, natural history, and the proposed mechanisms of epileptogenisis in FCD. Advances in neuroimaging techniques have enhanced the recognition of this pathology, which can be subtle. Illustrative neurophysiology and imaging examples are provided to help the clinicians identify diagnostic evidence of suspected FCD. Given the refractory course to pharmacologic management, alternative options such as ketogenic diet, resective surgery or neuromodulation can be considered. Recognition of FCD pathology in children with early onset epilepsy should prompt timely evaluations for resective surgery, which may render a significant number of patients seizure-free and improve neurocognitive outcome., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Purpose: Gorham-Stout disease (GS) is a rare and little understood bone disease that leads to the progressive replacement of the affected bone by fibrous tissues. The mandible is the most commonly affected craniofacial bone, but there is no report to date of temporomandibular joint (TMJ) lesions in this condition. We aimed to characterize the involvement of the TMJ in this uncommon bone disorder., Material and Methods: We retrospectively included all patients managed for craniofacial forms of GS in our pediatric institution over a period of 12 years. We collected clinical data on TMJ function and radiological data from computed tomography and magnetic resonance imaging., Results: Four pediatric patients were managed for craniofacial forms of GS between 2005 and 2017. All patients presented with various radiological lesions of the TMJs, including osteolytic lacunae of the condylar head, condylar head flattening and thinning of the glenoid cavity. Only one patient presented with functional TMJ impairment., Conclusion: The TMJ appears to be radiology affected in pediatric craniofacial forms of GS. Nevertheless, TMJ lesions, even when radiologically severe, rarely impair TMJ function. TMJ structure and function should be systematically assessed in craniofacial forms of GS, and, in the case of joint lesions, a regular follow-up of TMJ function should be considered., (Copyright © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Purpose: To use multimodal magnetic resonance imaging (MRI) to quantify treatment-induced changes in the whole volume of diffuse infiltrating pontine gliomas and correlate them with progression-free survival (PFS)., Methods and Materials: This prospective study included 22 children aged 3.3 to 14.7 years (median, 5.9 years). Multimodal MRI was performed at 3 distinct time points: before treatment, the first week following radiation therapy (RT), and 2 months after RT. The imaging protocol included morphologic, multi b-value diffusion; arterial spin labeling; and dynamic susceptibility contrast-enhanced perfusion. Morphologic and multimodal data-lesion volume, diffusion coefficients, relative cerebral blood flow, and relative cerebral blood volume (rCBV)-were recorded at the 3 aforementioned time points. The Wilcoxon test was used to compare each individual parameter variation between time points, and its correlation with PFS was assessed by the Spearman test., Results: Following RT, the tumors' solid component volume decreased by 40% (P<.001). Their median diffusion coefficients decreased by 20% to 40% (P<.001), while median relative cerebral blood flow increased by 60% to 80% (P<.001) and median rCBV increased by 70% (P<.001). PFS was positively correlated with rCBV measured immediately after RT (P=.003), and in patients whose rCBV was above the cutoff value of 2.46, the median PFS was 4.6 months longer (P=.001). These indexes tended to return to baseline 2 months after RT. Lesion volume before or after RT was not correlated with survival., Conclusions: Multimodal MRI provides useful information about diffuse infiltrating pontine gliomas' response to treatment; rCBV increases following RT, and higher values are correlated with better PFS. High rCBV values following RT should not be mistaken for progression and could be an indicator of response to therapy., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Epilepsy is a paroxysmal condition characterized by repeated transient seizures separated by longer interictal periods. Ictogenesis describes the processes of transition from the interictal state to a seizure. The processes include a preictal state, with specific clinical signs and a distinct electrophysiology which may provide opportunities to anticipate, or even prevent, seizures. Biological mechanisms of ictogenesis remain poorly understood and may vary between conditions/syndromes. We review here ictogenic processes including the involvement of pyramidal cells, interneurons and astrocytes, GABAergic and glutamatergic signaling, and ionic perturbations. Our review suggests that specific excitatory influences at the transition to an ictal event include (1) GABA receptor activation with a neuronal Cl(-) load and (2) a transient increase in external K(+)., (© 2014 Elsevier Inc. All rights reserved.)

Background: In cryptogenic epilepsy or when multifocal seizure onset is suspected, intracranial monitoring of the EEG is required., Objective: To report on the adverse events related to electroencephalogram (EEG) intracranial recording in one of the largest pediatric series published and to discuss the avoidance of adverse events in our experience and with respect to a review of the literature., Methods: A retrospective analysis of our department database and hospital charts of 95 children operated on between 1994 and 2009 was performed., Results: Invasive recording was uneventful in 51.1% of cases. Observed frequency of infection was 14.9%, cerebrospinal fluid leak was 10.6%, brain swelling was 6.4%, and hemorrhage was 17%. Brain swelling was more frequent in older patients, whereas the length of recording, number of electrode contacts used, and presence of depth electrodes were not relevant. Cerebrospinal fluid leakage was completely prevented by the routine introduction of dural graft substitutes in 2003., Conclusion: Invasive recordings carry a noticeable rate of adverse events but provide invaluable information in delineating the epileptogenic zone. The low incidence of such events among younger children suggests that invasive recordings can be successfully performed with low morbidity in this age group.

Background: Subdural collection is a possible adverse event that can occur after hemispherotomy., Objective: : We describe our surgical technique to avoid subdural collection after peri-insular hemispherotomy in children., Methods: Since 2008, we have closed the corticotomy with interrupted pial sutures and fibrinogen glue in 16 patients., Results: This operative technique stopped the occurrence of postoperative subdural collection and was not associated with an increased incidence of postoperative hydrocephalus., Conclusion: We recommend closing ventricular access after functional hemispherotomy, notably in patients who have had a stroke.