BACKGROUND AND OBJECTIVES:Subependymal giant cell astrocytomas (SEGA) are low-grade intraventricular tumors generally associated with tuberous sclerosis complex. Although mechanistic target of rapamycin inhibitors are recommended as first-line therapy for progressive SEGA without hydrocephalus, surgery is still relevant because 40% of patients are drug resistant. This study aimed to evaluate surgical outcomes in terms of tumor control, complication rates, and ventriculoperitoneal shunt (VPS) requirement, through a single-center retrospective series and a meta-analysis. METHODS:We retrospectively analyzed 31 pediatric SEGA resections performed over 2 decades at a tertiary care center. Clinical, radiological, and surgical variables were examined to identify predictors of outcome. In addition, a systematic literature review and meta-analysis were conducted to contextualize our findings. RESULTS:Gross total resection was achieved in 64.5% of cases, with a 19.3% rate of transient postoperative complications and a 6.4% incidence of VPS placement. No permanent morbidity or mortality occurred. Favorable outcome was associated with a smaller tumor volume and absence of preoperative hydrocephalus. The meta-analysis confirmed that preoperative hydrocephalus was the main predictor of postoperative complications (odds ratio 2.30) and shunt dependence (odds ratio 3.45). Five-year progression-free survival was 90.9% in the gross total resection subgroup and 100% in patients with unilateral tumors. Bilateral tumor location was an independent predictor of recurrence (hazard ratio = 17.79, P = .02). CONCLUSION:Microsurgical resection of SEGA is a safe and effective therapeutic option, particularly in patients without hydrocephalus. Early surgical intervention may reduce the need for VPS and long-term complications, offering a valid alternative or complement to mechanistic target of rapamycin inhibitor therapy, with durable tumor control especially in unilateral cases.

Pediatric brain arteriovenous malformation (PbAVM) is a potentially life-threatening condition accounting for intracerebral hemorrhage and other neurological complications. Our study aimed to elucidate the relationships between PbAVM angioarchitecture, spatial distribution, and clinical presentation. This retrospective study included all consecutive PbAVMs managed in a tertiary pediatric center (2007–2023). We performed a hierarchical clustering based on quantitative imaging data and constructed a 3D MRI-based atlas. We derived and analyzed clusters for initial presentation, clinical parameters (cure, recurrent bleeding, proliferation/recurrence, seizures) and location. We included 245 pediatric brain arteriovenous malformations in 234 patients (74.8

We aimed to decipher the natural history of paediatric cerebral and intraspinal medullary cavernous malformations and their genetic background, with an emphasis on the effect of mutational burden on their radiological features and haemorrhagic risk. We retrospectively collected clinical and radiological data and the natural history of the disease during the follow-up of all consecutive patients with cavernous malformations over the last 2 decades at a unique paediatric centre. We created an MRI atlas after lesion segmentation and normalization to perform voxel-based lesion symptom mapping. Targeted germline DNA sequencing of CCM1-3 genes and somatic lesions and targeted sequencing of RAS and PI3 K pathway genes were performed. In total, 257 patients carrying 786 brain and 14 spinal cavernous malformations were analysed. Age at diagnosis was 9.5 ± 5 years. Follow-up data were available for 718 lesions with a mean follow-up of 5.59 ± 3.28 years. Then, 134 brain (80 with focal onset seizures) and 10 spinal cavernous malformations were surgically excised. Furthermore, 95 of the/144 DNA extracted from lesions were successfully sequenced for somatic variants (PIK3CA, MAP3K3, KRIT1 and KRAS). We found an annual haemorrhagic risk of 1.9%, which was increased by type (sporadic or postradiation), location (brainstem or spinal), and radiological parameters (extracapsular haemorrhage, volume, signal, or presence of developmental venous anomaly). Cavernous malformations symptoms and radiological features were highly dependent on brain location. Surgical risk for cortical brain lesions was 0.9%, with 85% international league against epilepsy score grade of 1-2. Furthermore, 62% of operated lesions carried a pathogenic PIK3CA variant. The MAP3K3 somatic variant alone or in association with a PIK3CA variant decreased the annual haemorrhagic risk by 4.8 ± 1.5 (P < 0.05). We confirmed that 96% of cavernous malformations that are satellites of a developmental venous anomaly carried a PIK3CA variant and increased the relative haemorrhagic risk by 3 ± 0.31 (P = 3.8.10-3). We report an annual haemorrhagic risk of 1.9%, which is modulated by radiological features (i.e. extracapsular haemorrhage, volume, signal, and location) and lesion type. Here, we describe the first MRI atlas. Surgery yields excellent general outcomes in supratentorial superficial lesions, unlike deep-seated cavernous malformations, which is associated with high morbidity. Molecular diagnosis of operated lesions highlighted PIK3CA as a factor associated with aggressiveness in both sporadic and familial cavernous malformations. MAP3K3 decreased the risk. Finally, we confirmed that developmental venous anomaly is a radiological landmark of PIK3CA.

OBJECTIVE:Intracranial empyemas (IEs) are rare but severe complications of ear, nose, and throat (ENT) infections, defined as purulent collections in the normally virtual meningeal spaces. IEs can be classified as sinogenic (S-IE) or otogenic (O-IE), according to the initial infection. These life-threatening complications can entail long-term neurological disabilities. Few studies have described their characteristics, and cohorts are usually small and heterogeneous. In the Greater Paris area, all cases of pediatric IE are referred to the authors' center. The aim of this study was to describe the characteristics of IE and their treatment and risk factors for repeated surgery and neurological disabilities. METHODS:This single-center, retrospective observational cohort study included all consecutive cases of IE admitted to the authors' institution between January 2016 and January 2025. RESULTS:A total of 248 patients with IE were included: 115 with S-IE and 133 with O-IE. The mean age was 6.9 ± 5.2 years. An increase in the incidence of IEs was observed over the final years of the study. Two clinical presentations were distinguished. O-IEs were observed in younger children (mean 40 ± 37 months vs 133 ± 44 months for S-IE). O-IEs were almost exclusively extradural (96%), whereas around half of S-IEs had subdural involvement (51%). S-IEs were associated with more neurological symptoms. All patients underwent surgery. Overall, 95% of patients with O-IEs and 85% of those with S-IEs underwent ENT surgery. Neurosurgery was necessary for 7% (9/133) of O-IEs and 60% (69/115) of S-IEs. Bacteriological samples were obtained, and identification was achieved in 86% (culture or polymerase chain reaction techniques). For O-IE, Fusobacterium necrophorum (47%) was the most frequent, whereas in S-IE cases, the Streptococcus milleri group (52%) was predominant. Cerebral venous thrombosis (CVT) was the most frequent complication, in 55% of O-IE cases and 21% of S-IE cases (p < 0.0001). There were no deaths in this cohort. Neurological disabilities were observed in 17% of patients, with a higher rate for those with S-IEs (27%) than those with O-IEs (8%) (p < 0.0001). Subdural empyema and parietal and interhemispheric locations were identified risk factors for neurological sequelae. Repeat surgery was necessary for 30% of patients with S-IEs and 11% of patients with O-IEs (p < 0.0001). The observed risk factors were residual IE at control MRI and the association of CVT. CONCLUSIONS:This study describes the largest pediatric IE cohort to date. An increase in incidence has been observed since 2021. The favorable results of this cohort suggest that the authors' multidisciplinary standardized treatment protocol provides successful treatment to most patients with IE.

INTRODUCTION:Laser Interstitial Thermal Therapy under MRI control has emerged as a safe and efficient alternative to microsurgery in epilepsy and neurooncology procedures. Yet it has been used only recently in seldom European centers. Here, we report our 4 years' experience with LITT in children (complications, epileptic and oncologic outcomes). METHODS:Single center retrospective study of consecutive LITT procedures during the 2021-2024 period. LITT was restricted to pediatrics in the following indications: (i) circumscribed lesion (ii) benign pathology (iii) problematic location for microsurgical resection. After a robotic stereotactic implantation, ablation was performed under MRI guidance with the VISUALASE system. Post operative outpatient clinic and MRI were systematic at 1, 3, 6 and 12 months. Epileptic outcome was defined after ILAE classification, oncological relapse was defined as a volumetric increase after 1 month of follow up. RESULTS:35 children (mean age 11.4yo, M/F ratio = 0.8) were operated in 41 successive procedures. Main indication was focal epilepsy (n = 28 patients; FCD n = 13, LEAT n = 11, HH n = 4) while 7 children were treated for oncological purposes. Lesions were cortical in the insula (n = 8), mediotemporobasal (n = 8) and paracentral (n = 6) regions or subcortical (hypothalamus and basal ganglia n = 6, mesencephalon n = 4). Perioperative transient adverse events occurred in 25.7% and persistent neurological deficit was noted in 2 children. After a mean follow up of 31.2 months, 67.9% of the patients are seizure free and tumoral recurrence was observed in 3/18 cases. CONCLUSION:In this pediatric cohort of 35 children with challenging brain lesions, LITT ablation was safe and efficient.

Focal cortical dysplasia, hemimegalencephaly and cortical tubers are paediatric epileptogenic malformations of cortical development (MCDs) frequently pharmacoresistant and mostly treated surgically by the resection of epileptic cortex. Availability of cortical resection samples has allowed significant mechanistic discoveries directly from human material. Causal brain somatic or germline mutations in the AKT/PI3K/DEPDC5/MTOR genes have been identified. GABA(A)-mediated paradoxical depolarization, related to altered chloride (Cl-) homeostasis, has been shown to participate to ictogenesis in human paediatric MCDs. However, the link between genomic alterations and neuronal hyperexcitability is unclear. Here, we studied the post-translational interactions between the mTOR pathway and the regulation of cation-chloride cotransporters (CCCs), KCC2 and NKCC1, that are largely responsible for controlling intracellular Cl- and, ultimately, GABAergic transmission. For this study, 35 children (25 MTORopathies and 10 pseudo-controls, diagnosed by histology plus genetic profiling) were operated for drug-resistant epilepsy. Postoperative cortical tissues were recorded on a multi-electrode array to map epileptic activities. CCC expression level and phosphorylation status of the WNK1/SPAK-OSR1 pathway was measured during basal conditions and after pharmacological modulation. Direct interactions between mTOR and WNK1 pathway components were investigated by immunoprecipitation. Membranous incorporation of MCD samples in Xenopus laevis oocytes enabled measurement of the Cl- conductance and equilibrium potential for GABA. Of the 25 clinical cases, half harboured a somatic mutation in the mTOR pathway, and pS6 expression was increased in all MCD samples. Spontaneous interictal discharges were recorded in 65% of the slices. CCC expression was altered in MCDs, with a reduced KCC2/NKCC1 ratio and decreased KCC2 membranous expression. CCC expression was regulated by the WNK1/SPAK-OSR1 kinases through direct phosphorylation of Thr(906) on KCC2, which was reversed by WNK1 and SPAK antagonists (N-ethylmaleimide and staurosporine). The mSIN1 subunit of MTORC2 was found to interact with SPAK-OSR1 and WNK1. Interactions between these key epileptogenic pathways could be reversed by the mTOR-specific antagonist rapamycin, leading to a dephosphorylation of CCCs and recovery of the KCC2/NKCC1 ratio. The functional effect of such recovery was validated by the restoration of the depolarizing shift in the equilibrium potential for GABA by rapamycin, measured after incorporation of MCD membranes into X. laevis oocytes, in line with a re-establishment of normal Cl- reversal potential. Our study deciphers a protein interaction network through a phosphorylation cascade between MTOR and WNK1/SPAK-OSR1 leading to deregulation of chloride cotransporters, increased neuronal Cl- levels and GABA(A) dysfunction in malformations of cortical development, linking genomic defects and functional effects and paving the way to target epilepsy therapy.

OBJECTIVE:Guidelines for the management of pediatric severe traumatic brain injury (TBI) recommend external ventricular drainage for CSF drainage as a first-tier treatment in the intracranial pressure (ICP) pathway. However, ventriculostomy in children can sometimes be challenging because of the small size of the lateral ventricles. External lumbar drainage (ELD) may be a useful alternative; therefore, the authors analyzed the outcome of a cohort of pediatric patients who underwent ELD to manage intracranial hypertension (ICH). METHODS:This study retrospectively enrolled pediatric patients with ICH following severe TBI who underwent ELD. Radiological and clinical severity scores (Marshall classification, Rotterdam score, Injury Severity Score, and Pediatric Trauma Score) were noted. ICP and cerebral perfusion pressure (CPP) curves were analyzed 12 hours before and after the procedure. Any change in medical therapy was recorded, as well as the total volume and duration of drainage. Cerebellar tonsillar position according to the McRae line was noted before and after ELD. Glasgow Outcome Scale-Extended score at follow-up was also noted. RESULTS:Thirty patients were included, with a mean age of 8 ± 4.4 years, and a median admission Glasgow Coma Scale score of 7 ± 4 (range 3-13). ELD was performed after a median delay of 1 day (range 0-7 days), mean drainage volume/day was 296 ± 129 ml, and median duration of drainage was 7 ± 5 (range 2-12) days. Forty-three percent of the patients underwent ELD as a part of the first-tier therapy. ICP decreased after ELD (mean difference 13.4 ± 6.2 mm Hg, p < 0.001), whereas CPP increased (mean difference 10.6 ± 6.4 mm Hg, p < 0.001). Fifty-three percent of the cohort did not need any further second-tier therapy after ELD. The study found 1 case of drain revision and 3 cases of cerebellar tonsil herniation. CONCLUSIONS:These preliminary data suggest ELD is a valuable option to treat ICH in severely head-injured children, limiting the use of second-tier treatments. This pilot study should lay the foundation for a multicenter prospective trial.

OBJECTIVE:To describe and compare clinical and microbiological features, surgical and medical management, and outcomes of children with otogenic and sinogenic intracranial empyema (IE) in an institution with an established multidisciplinary protocol. To use the study findings to inform and update the institutional algorithm. METHODS:Retrospective analysis was carried out on the electronic healthcare records of all children with oto-sinogenic IE admitted in a 5-year period. RESULTS:A total of 76 patients were identified and treated according to an institutional protocol. Two distinct groups were identified: intracranial empyema related to otogenic infection (OI-IE, n = 36) or sinogenic infection (SI-IE, n = 40). SI-IE was seen in older children and had a significantly higher morbidity. Sub-dural IE was seen in a minority (n = 16) and only in SI-IE and required urgent collaborative ENT-neurosurgery. Extra-dural IE occurred more frequently and was seen in both SI-IE and OI-IE. No death and overall low morbidity were observed. Particularities found in SI-IE and OI-IE groups (as thrombosis, microbiology, antibiotic treatment, duration and outcome) permitted the delineation of these groups in our updated algorithm. CONCLUSION:The presence of a collaborative multidisciplinary protocol permits the step-wise co-ordination of care for these complex patients in our institution. All patients received prompt imaging, urgent surgical intervention, and antibiotic treatment. Microbiological identification was possible for each patient and antibiotic rationalization was permitted through use of Polymerase chain reaction (PCR) testing in cases of sterile cultures. Of note, intracranial empyema related to sinogenic infection is shown to have significantly more severe clinical presentation, a higher morbidity, and a longer duration of antibiotic therapy than that related to otogenic infection. Study findings allowed for the update and clarification of the institutional protocol, which now clearly demarcates the clinical presentation, biological evidence, radiology, surgical and medical treatments in children with oto-sinogenic IE.

OBJECTIVE:The outcome of endoscopic third ventriculostomy (ETV) in children who had previously received shunts and who were experiencing shunt dysfunction is still discussed in terms of efficacy (success rate from 40% to 80%) and safety (0%-32.5% of complications). Reported predictive factors of secondary ETV failure are age, early onset of hydrocephalus, and prematurity. The best surgical strategy in the different subgroups of patients with shunt dysfunction is still debated. Therefore, the authors aimed to identify subgroups of patients in whom shunt treatment was associated with favorable outcome of ETV, to define the role of ETV in patients with global rostral midbrain dysfunction syndrome. METHODS:This study was a monocentric retrospective case series and a meta-analysis of children who had previously received shunts and who underwent secondary ETV for shunt dysfunction between 2012 and 2022. Clinical and MRI features were examined, along with surgical outcome, etiology of hydrocephalus, and preoperative ETV Success Score. Univariate and multivariate analyses were performed to find predictors of outcome of secondary ETV. Youden's J index was calculated on age distribution to find an optimal age cutoff. Systematic review of the literature and a meta-analysis were performed according to the PRISMA statement. RESULTS:Seventy consecutive patients were included. The overall success rate of secondary ETV was 63%. Primary obstructive hydrocephalus, age ≥ 36 months, and the presence of aqueductal obstruction were predictors of ETV success. Multivariate analysis found that age < 36 months, primary inflammatory hydrocephalus, and presence of fourth ventricular obstruction were associated with ETV failure. All patients with global rostral midbrain dysfunction syndrome experienced clinical and radiological improvement after ETV. The meta-analysis showed that postinflammatory etiology and age < 36 months were predictors of ETV failure. CONCLUSIONS:ETV is safe and effective for children with obstructive hydrocephalus experiencing shunt dysfunction, notably in cases of primary obstructive hydrocephalus with aqueductal stenosis, and among children whose age was ≥ 36 months who had postinflammatory hydrocephalus.

Periodic discharges are a rare peculiar electroencephalogram pattern, occasionally associated with motor or other clinical manifestations, usually observed in critically ill patients. Their underlying pathophysiology remains poorly understood. Epileptic spasms in clusters and periodic discharges with motor manifestations share similar electroencephalogram pattern and some aetiologies of unfavourable prognosis such as subacute sclerosing panencephalitis or herpes encephalitis. Arterial spin labelling magnetic resonance imaging identifies localizing ictal and inter-ictal changes in neurovascular coupling, therefore assumed able to reveal concerned cerebral structures. Here, we retrospectively analysed ictal and inter-ictal arterial spin labelling magnetic resonance imaging in patients aged 6 months to 15 years (median 3 years 4 months) with periodic discharges including epileptic spasms, and compared these findings with those of patients with drug-resistant focal epilepsy who never presented periodic discharges nor epileptic spasms as well as to those of age-matched healthy controls. Ictal electroencephalogram was recorded either simultaneously with arterial spin labelling magnetic resonance imaging or during the close time lapse of patients' periodic discharges, whereas inter-ictal examinations were performed during the patients' active epilepsy but without seizures during the arterial spin labelling magnetic resonance imaging. Ictal arterial spin labelling magnetic resonance imaging was acquired in five patients with periodic discharges [subacute sclerosing panencephalitis (1), stroke-like events (3), West syndrome with cortical malformation (1), two of them also had inter-ictal arterial spin labelling magnetic resonance imaging]. Inter-ictal group included patients with drug-resistant epileptic spasms of various aetiologies (14) and structural drug-resistant focal epilepsy (8). Cortex, striatum and thalamus were segmented and divided in six functional subregions: prefrontal, motor (rostral, caudal), parietal, occipital and temporal. Rest cerebral blood flow values, absolute and relative to whole brain, were compared with those of age-matched controls for each subregion. Main findings were diffuse striatal as well as cortical motor cerebral blood flow increase during ictal examinations in generalized periodic discharges with motor manifestations (subacute sclerosing panencephalitis) and focal cerebral blood flow increase in corresponding cortical-striatal-thalamic subdivisions in lateralized periodic discharges with or without motor manifestations (stroke-like events and asymmetrical epileptic spasms) with straight topographical correlation with the electroencephalogram focus. For inter-ictal examinations, patients with epileptic spasms disclosed cerebral blood flow changes in corresponding cortical-striatal-thalamic subdivisions (absolute-cerebral blood flow decrease and relative-cerebral blood flow increase), more frequently when compared with the group of drug-resistant focal epilepsies, and not related to Vigabatrin treatment. Our results suggest that corresponding cortical-striatal-thalamic circuits are involved in periodic discharges with and without motor manifestations, including epileptic spasms, opening new insights in their pathophysiology and new therapeutical perspectives. Based on these findings, we propose a model for the generation of periodic discharges and of epileptic spasms combining existing pathophysiological models of cortical-striatal-thalamic network dynamics. Eisermann et al. report from arterial spin labelling magnetic resonance imaging studies in children that cortico-striatal-thalamic circuits are involved in periodic electroencephalogram discharges and epileptic spasms and propose a model for their generation by combining existing pathophysiological models of cortico-striatal-thalamic network dynamics.

BACKGROUND AND OBJECTIVES: When seizure onset affects a whole hemisphere, hemispheric disconnections are efficient and safe procedures. However, both lateral peri-insular hemispherotomy and vertical paramedian hemispherotomy approaches report a failure rate around 20%, which can be explained by residual connections giving rise to persistent seizures. In this study, we present the interhemispheric vertical hemispherotomy (IVH), a technical variation of the vertical paramedian hemispherotomy approach, that aims to increase seizure control avoiding residual connections while exposing the corpus callosum. METHODS: This is a retrospective study of IVH in two centers, with analysis of clinical and MRI data and outcomes. A detailed description of the technique is provided with a video. RESULTS: IVH was performed in 39 children. The mean age at surgery was 7.2 years, and etiologies were as follows: malformations of cortical development (n = 14), Rasmussen's encephalitis (n = 10), stroke (n = 10), post-traumatic (3), and Sturge-Weber Syndrome (2). Hemispheric disconnection was complete on postoperative MRI in 34 cases. There was no mortality, hydrocephalus occurred in one case, and subdural collection occurred in four cases. A second surgery was performed in four cases because of seizure relapse (n = 3) and/or incomplete disconnection on MRI (n = 4). With a mean follow-up of 3.2 years, International League Against Epilepsy class I epilepsy outcome was obtained for 37/39 patients. CONCLUSION: IVH is a safe and effective variation of the vertical approaches for hemispheric disconnection. It allows a good exposure and anatomic control of the corpus callosum, which is a frequent site of incomplete disconnection. IVH may be limited by the thalamic volume and the ventricular size, notably in hemimegalencephaly cases.

Zolpidem is a sedative drug that has been shown to induce a paradoxical effect, restoring brain function in wide range of neurological disorders. The underlying functional mechanism of the effect of zolpidem in the brain in clinical improvement is still poorly understood. Thus, we aimed to investigate rest brain function to study zolpidem-induced symptom improvement in a patient who developed postoperative pediatric cerebellar mutism syndrome, a postoperative complication characterized by delayed onset transient mutism/reduced speech that can occur after medulloblastoma resection. The patient experienced clinical recovery after a single dose of zolpidem. Brain function was investigated using arterial spin labeling MRI and resting-state functional MRI. Imaging was performed at three time-points: preoperative, postoperative during symptoms, and after zolpidem intake when the symptoms regressed. Whole brain rest cerebral blood flow (CBF) and resting state functional connectivity using Pearson coefficient correlations between pairs of regions of interest were investigated two-by-two at the different time points. A comparison between postoperative and preoperative images showed a significant decrease in rest CBF in the left supplementary motor area, Broca’s area, and the left striatum and a decrease in functional connectivity within the dentato-thalamo-cortical and cortico-striato-pallido-thalamo-cortical loops. Post-zolpidem images showed increased CBF in the left striatum and increased functional connectivity within the disrupted loops relative to postoperative images. Thus, we observed functional changes within the broader speech network and thalamo-subcortical interactions associated with the paradoxical effect of zolpidem in promoting clinical recovery. This should encourage further functional investigations in the brain to better understand the mechanism of zolpidem in neurological recovery.

CONTEXT:Endocrine complications are common in pediatric brain tumor patients.OBJECTIVE:To describe hypothalamic-pituitary-gonadal axis (HPGA) function in patients treated in childhood for a primary brain tumor more than 5 years earlier, in order to identify risk factors for HPGA impairment.METHODS:We retrospectively included 204 patients diagnosed with a primary brain tumor before 18 years of age and monitored at the pediatric endocrinology unit of the Necker Enfants-Malades University Hospital (Paris, France) between January 2010 and December 2015. Patients with pituitary adenoma or untreated glioma were excluded.RESULTS:Among patients with suprasellar glioma not treated by radiotherapy, the prevalence of advanced puberty was 65% overall and 70% when the diagnosis occurred before 5 years of age. Medulloblastoma chemotherapy caused gonadal toxicity in 70% of all patients and in 87.5% of those younger than 5 years at diagnosis. In the group with craniopharyngioma, 70% of patients had hypogonadotropic hypogonadism, which was consistently accompanied by growth hormone deficiency.CONCLUSION:Tumor type, location, and treatment were the risk main factors for HPGA impairment. Awareness that onset can be delayed is essential to guide information of parents and patients, patient monitoring, and timely hormone replacement therapy.

OBJECTIVE Epidural hematoma (EDH) has rarely been studied specifically in infants. The objective of this study was to investigate the outcomes of patients aged < 18 months (infants) with EDH. METHODS The authors conducted a single-center retrospective study of 48 infants aged less than 18 months who underwent an operation for a supratentorial EDH in the last decade. Clinical, radiological, and biological variables were used in a statistical analysis to identify factors predictive of radiological and clinical outcome. RESULTS Forty-seven patients were included in the final analysis. Seventeen children (36%) had cerebral ischemia on postoperative imaging, either due to stroke (cerebral herniation) or by local compression. Factors associated with ischemia after multivariate logistic regression were the presence of an initial neurological deficit (76% vs 27%, p = 0.03), low platelet count (mean 192 vs 267 per mm3, p = 0.01), low fibrinogen level (mean 1.4 vs 2.2 g/L, p = 0.04) and long intubation time (mean 65.7 vs 10.1 hours, p = 0.03). Cerebral ischemia on MRI was predictive of a poor clinical outcome. CONCLUSIONS Infants with EDH have a low mortality rate but a high risk of cerebral ischemia, along with long-term neurological sequelae.

BACKGROUND: Focal cortical dysplasia (FCD) causes drug-resistant epilepsy in children that can be cured surgically, but the lesions are often unseen by imaging. OBJECTIVE: To assess the efficiency of arterial spin labeling (ASL), voxel-based-morphometry (VBM), fMRI electroencephalography (EEG), resting-state regional homogeneity (ReHo), 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET), and their combination in detecting pediatric FCD. METHODS: We prospectively included 10 children for whom FCD was localized by surgical resection. They underwent 3T MR acquisition with concurrent EEG, including ASL perfusion, resting-state BOLD fMRI (allowing the processing of EEG-fMRI and ReHo), 3D T1-weighted images processed using VBM, and FDG PET-CT coregistered with MRI. Detection was assessed visually and by comparison with healthy controls (for ASL and VBM). RESULTS: Eight children had normal MRI, and 2 had asymmetric sulci. Using MR techniques, FCD was accurately detected by ASL for 6/10, VBM for 5/10, EEG-fMRI for 5/8 (excluding 2 with uninterpretable results), and ReHo for 4/10 patients. The combination of ASL, VBM, and ReHo allowed correct FCD detection for 9/10 patients. FDG PET alone showed higher accuracy than the other techniques (7/9), and its combination with VBM allowed correct FCD detection for 8/9 patients. The detection efficiency was better for patients with asymmetric sulci (2/2 for all techniques), but advanced MR techniques and PET were useful for MR-negative patients (7/8). CONCLUSION: A combination of multiple imaging techniques, including PET, ASL, and VBM analysis of T1-weighted images, is effective in detecting subtle FCD in children.

BACKGROUND:Focal cortical dysplasias (FCD) are a frequent cause of drug-resistant epilepsy in children but are often undetected on structural magnetic resonance imaging (MRI). We aimed to measure and validate the variation of resting state functional MRI (rs-fMRI) blood oxygenation level dependent (BOLD) metrics in surgically proven FCDs in children, to assess the potential yield for detecting and understanding these lesions.METHODS:We prospectively included pediatric patients with surgically proven FCD with inconclusive structural MRI and healthy controls, who underwent a ten-minute rs-fMRI acquired at 3T. Rs-fMRI data was pre-processed and maps of values of regional homogeneity (ReHo), degree centrality (DC), amplitude of low frequency fluctuations (ALFF) and fractional ALFF (fALFF) were calculated. The variations of BOLD metrics within the to-be-resected areas were analyzed visually, and quantitatively using lateralization indices. BOLD metrics variations were also analyzed in fluorodeoxyglucose-positron emission tomography (FDG-PET) hypometabolic areas.RESULTS:We included 7 patients (range: 3-15 years) and 6 aged-matched controls (range: 6-17 years). ReHo lateralization indices were positive in the to-be-resected areas in 4/7 patients, and in 6/7 patients in the additional PET hypometabolic areas. These indices were significantly higher compared to controls in 3/7 and 4/7 patients, respectively. Visual analysis revealed a good spatial correlation between high ReHo areas and MRI structural abnormalities (when present) or PET hypometabolic areas. No consistent variation was seen using DC, ALFF, or fALFF.CONCLUSION:Resting-state fMRI metrics, noticeably increase in ReHo, may have potential to help detect MRI-negative FCDs in combination with other morphological and functional techniques, used in clinical practice and epilepsy-surgery screening.

BACKGROUND: Hydrocephalus frequently occurs with midline posterior fossa cystic collections. The classification of this heterogeneous group of developmental anomalies, including Dandy–Walker malformation, persisting Blake's pouch, retrocerebellar arachnoid cysts, and mega cisterna magna, is subject of debate. The absence of diagnostic criteria is confusing regarding the ideal management of PFCC-related hydrocephalus. OBJECTIVE: To decipher the surgical strategy for the treatment of children with PFCC-related hydrocephalus through a retrospective analysis of the surgical outcome driven by their clinical and radiological presentation. METHODS: This study enrolled patients operated of symptomatic PFCC-related hydrocephalus. Clinical and MRI features were examined, as well as the surgical outcome. Unbiased subgroup classification of the patients was performed with multiple component analysis as a function of imaging characteristics and hierarchical clustering on principal component. Outcome was assessed with binomial logistic regression and Kaplan–Meier analysis. RESULTS: Fifty-four patients were included between 2007 and 2021. Multiple component analysis suggested that cerebellar and vermian hypoplasia, vermian rotation, basal–tentorial angle, and fastigial angle were strongly correlated. Hierarchical clustering and the distribution of the patients in the bidimensional plot showed the clear segregation of 3 major clusters, which correlated with the radiological diagnosis (P < .01). Binomial logistic regression and survival analysis showed that endoscopic third ventriculostomy was an effective treatment for patients with persisting Blake's pouch, while failing to control hydrocephalus in most of patients with Dandy–Walker malformation. CONCLUSION: Preoperative MRI in patients with PFCC-related hydrocephalus is essential to better define the diagnosis. The choice of treatment strategy notably relies on correct radiological diagnosis.

BackgroundThe co-occurrence of moyamoya vasculopathy and extra-optic pathway tumors is rare in neurofibromatosis type 1 (NF1), with only four cases described in the literature. Brain surgery in these patients may be challenging because of the risk of brain infarction after skin and dural incision. Given its percutaneous and minimally invasive nature, laser interstitial thermal therapy (LITT) is an ideal option for the treatment of brain tumors in these patients. Here, we report on two patients with NF1 and moyamoya syndrome (MMS) treated for a brain glioma with LITT, after cerebral revascularization.CasesThe first patient, with familial NF1, underwent bilateral indirect revascularization with multiple burr holes (MBH) for symptomatic MMS. Two years later, she was diagnosed with a left temporal tumor, with evidence of radiologic progression over 10 months. The second patient, also with familial NF1, developed unilateral MMS when he was 6 years old and was treated with MBH. At the age of 15 years, MRI showed a right cingular lesion, growing on serial MRIs. Both patients underwent LITT with no perioperative complications; they are progression free at 10 and 12 months, respectively, and the tumors have decreased in volume.DiscussionWhile the association of extra-optic neoplasm and moyamoya angiopathy is seldom reported in NF1, tumor treatment is challenging in terms of both avoiding stroke and achieving oncological control. Here, we show in 2 cases, that LITT could be a safe and effective option in these rare conditions.

OBJECTIVE:The aim of this study was to determine the cognitive profile of children with a temporal arachnoid cyst (TAC) and its impact on daily life.METHODS:The authors prospectively analyzed the cognitive and psychological profiles of 100 consecutive children relative to age and cyst characteristics (side, cyst size, and cyst shape: convex or nonconvex) and their outcome 4 years later.RESULTS:Mean IQs were normal but with high heterogeneity on Full Scale IQ (FSIQ; range 59-150); 29% of children had at least one Wechsler index below the norm, in particular, Processing Speed and Working Memory Indexes. Impairments were observed in language for 31% of children, as well as in verbal memory (28%), visual memory (23%), executive function (21%), and visual attention (24%). Half of the children (50%) needed rehabilitation for learning difficulties, and 26% had academic difficulties. The parental questionnaire BRIEF (Behavior Rating Inventory of Executive Function) revealed significant executive dysfunctions in daily life for 22% of the children. One-third of the patients (34%) required psychotherapy for anxiety or social disorders, with higher rates in patients with a right-sided cyst and older children. Cyst size had very little neuropsychological impact. Convex cysts were significantly associated with worse performance than nonconvex cysts on all Wechsler indexes and FSIQ, and in language, verbal memory, attention, and visuospatial skills. Children with a convex cyst had significantly more executive and behavior difficulties in daily life and more psychotherapy than other children. The effect of cyst shape was independent of Galassi type and cyst side. Children with a ruptured cyst or an incidentally discovered cyst usually had a good cognitive level. Four years later, children without initial disorders remained stable, whereas those with difficulties who did not undergo surgery needed more rehabilitation and school adaptations.CONCLUSIONS:This large cohort study revealed a varied profile of children with a TAC: at initial assessment, 50% had neuropsychological difficulties and needed rehabilitation and/or psychotherapy for learning or behavior difficulties, and 50% had no difficulties, which may explain the debate about this pathology. Patients with neuropsychological difficulties had a heterogeneous profile with normal intelligence but selective cognitive and/or behavior disorders that may have a long-term impact on their quality of life, particularly those with a right-sided cyst. A neuropsychological evaluation is not always necessary for a cyst discovered incidentally, but early evaluation is essential in patients with academic, learning, or psychological disorders. When assessment shows selective disorders presumably linked to cyst location, surgery may be considered, particularly for convex cysts, as this study revealed more effects in association with cyst shape than with cyst size and significantly poorer performance with a convex cyst.