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1.
Int J Pediatr Otorhinolaryngol ; 124: 152-156, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31195309

RESUMO

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.

2.
Pediatrics ; 143(6)2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31068385

RESUMO

BACKGROUND: Our aim was to report the long-term clinical and imaging outcomes of ≤15-year-old children treated for ruptured or symptomatic cerebral aneurysms and to identify prognostic factors for clinical outcome, recurrence, and rebleeding. METHODS: We retrospectively identified all pediatric cases of cerebral aneurysm from 2000 to 2015 and then prospectively evaluated long-term occlusion using brain MRI and clinical outcome measures: outcome was considered favorable if King's Outcome Scale for Childhood Head Injury score was ≥5. We performed univariate analysis and logistic binary regression to identify variables associated with clinical and imaging outcomes. RESULTS: Fifty-one children (aged 8.5 ± 1.1 years [mean ± SD], with 37 ruptured and 14 symptomatic aneurysms) were included, and endovascular treatments (84%) or microsurgical procedures (16%) were performed. Despite a 19.6% death rate, at a mean follow-up of 8.3 years, 35 children (68.6%) had a favorable outcome. Annual bleeding and aneurysm recurrence rates were 1.4% ± 1.1% and 2.6% ± 1.8%, respectively. Cerebral ischemia, whether initial or delayed within the first month, was predictive of poor clinical outcome in multivariate analysis (odds ratio: 25; 95% confidence interval: 0.43-143; P < .0001), whereas aneurysm size >5 mm was the only factor associated with recurrence (odds ratio: 14.6; 95% confidence interval: 2.4-86.1; P = .003). CONCLUSIONS: Two-thirds of studied ≤15-year-old children suffering from ruptured or symptomatic cerebral aneurysms had long-term favorable outcome. Annual bleeding and aneurysm recurrence rates have shown to be low after endovascular or surgical treatment. Long-term imaging follow-up helps to depict aneurysm recurrence or de novo aneurysm formation and to prevent rebleeding.

3.
Am J Med Genet A ; 179(7): 1304-1309, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31004414

RESUMO

The spectrum of clinical consequences of variants in the Platelet derived growth factor receptor beta (PDGFRB) gene is wide. Missense variants leading to variable loss of signal transduction in vitro have been reported in the idiopathic basal ganglia calcification (IBGC) syndrome Type 4. In contrast, gain-of-function variants have been reported in infantile myofibromatosis, Penttinen syndrome, and Kosaki overgrowth syndrome. Here, we report a patient harboring a novel postzygotic variant in PDGFRB (c.1682_1684del, p.[Arg561_Tyr562delinsHis]) and presenting severe cerebral malformations, intracerebral calcifications, and infantile myofibromatosis. This observation expands the phenotype associated with PDGFRB variants and illustrates the wide clinical spectrum linked to dysregulation of PDGFRB.

4.
J Neurosurg Pediatr ; : 1-9, 2019 03 22.
Artigo em Inglês | MEDLINE | ID: mdl-30901748

RESUMO

OBJECTIVE: Arachnoid cysts (ACs) are most frequently located in the middle cranial fossa. Some patients are asymptomatic whereas others exhibit signs of increased intracranial pressure, seizures, or cognitive and behavioral symptoms. When ACs do require treatment, the optimal surgical technique remains controversial. This study was conducted to assess the most effective surgical treatment for these cysts. METHODS: The authors retrospectively reviewed 240 temporal intracranial ACs managed over a 25-year period in their pediatric neurosurgical unit. Pre- and posttreatment results were clinically and radiologically assessed. RESULTS: A majority of male patients (74.6%) with an overall median age of 6.9 years were included. The mean cyst size was 107 cm3; the Galassi classification showed 99 (41.3%) type I, 77 (32.1%) type II, and 64 (26.7%) type III cysts. Forty-four ACs (18.3%) were diagnosed after rupture. Surgical management was performed by microsurgery (28.3%), endoscopic cyst fenestration (14.6%), cystoperitoneal shunting (CPS; 16.2%), or subdural shunting (10%). Furthermore, 74 children (30.8%) did not undergo operations. After a mean follow-up of 4.1 years, the mean percentage decrease in cyst volume and the overall rate of clinical improvement did not significantly differ. The endoscopy group had earlier complications and a shorter event-free survival (EFS) time (EFS at 3 years = 67.7%, vs 71.5% and 90.5% for CPS and microsurgery, respectively; p < 0.007) and presented with more subdural hematomas compared to the microsurgery group (p < 0.005). The microsurgery group also showed a tendency for longer cystocisternostomy permeability than the endoscopy group. CONCLUSIONS: Concerning the management of unruptured symptomatic temporal ACs, microsurgery appears to be the most effective treatment, with longer EFS and fewer complications compared to shunting or endoscopy.

5.
Ann Neurol ; 85(2): 204-217, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30597612

RESUMO

OBJECTIVE: Dysregulation of γ-aminobutyric acidergic (GABAergic) transmission has been reported in lesional acquired epilepsies (gliomas, hippocampal sclerosis). We investigated its involvement in a developmental disorder, human focal cortical dysplasia (FCD), focusing on chloride regulation driving GABAergic signals. METHODS: In vitro recordings of 47 human cortical acute slices from 11 pediatric patients who received operations for FCD were performed on multielectrode arrays. GABAergic receptors and chloride regulators were pharmacologically modulated. Immunostaining for chloride cotransporter KCC2 and interneurons were performed on recorded slices to correlate electrophysiology and expression patterns. RESULTS: FCD slices retain intrinsic epileptogenicity. Thirty-six of 47 slices displayed spontaneous interictal discharges, along with a pattern specific to the histological subtypes. Ictal discharges were induced in proepileptic conditions in 6 of 8 slices in the areas generating spontaneous interictal discharges, with a transition to seizure involving the emergence of preictal discharges. Interictal discharges were sustained by GABAergic signaling, as a GABAA receptor blocker stopped them in 2 of 3 slices. Blockade of NKCC1 Cl- cotransporters further controlled interictal discharges in 9 of 12 cases, revealing a Cl- dysregulation affecting actions of GABA. Immunohistochemistry highlighted decreased expression and changes in KCC2 subcellular localization and a decrease in the number of GAD67-positive interneurons in regions generating interictal discharges. INTERPRETATION: Altered chloride cotransporter expression and changes in interneuron density in FCD may lead to paradoxical depolarization of pyramidal cells. Spontaneous interictal discharges are consequently mediated by GABAergic signals, and targeting chloride regulation in neurons may be considered for the development of new antiepileptic drugs. Ann Neurol 2019; 1-14 ANN NEUROL 2019;85:204-217.

6.
Ann Clin Transl Neurol ; 5(9): 1048-1061, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30250862

RESUMO

Objective: Preceding oxygen glucose deprivation (OGD) and ongoing seizures have both been reported to increase neuronal chloride concentration ([Cl-]i), which may contribute to anticonvulsant failure by reversing the direction of chloride currents at inhibitory GABAA synapses. Methods: The effects of OGD on [Cl-]i, seizure activity, and anticonvulsant efficacy were studied in a chronically epileptic in vitro preparation. Results: Seizures initially increased during OGD, followed by suppression. On reperfusion, seizure frequency and [Cl-]i progressively increased, and phenobarbital efficacy was reduced. Bumetanide (10 µmol/L) and furosemide (1 mmol/L) prevented or reduced the OGD induced [Cl-]i increase. Phenobarbital efficacy was enhanced by bumetanide (10 µmol/L). Furosemide (1 mmol/L) suppressed recurrent seizures. Interpretation: [Cl-]i increases after OGD and is associated with worsened seizure activity, reduced efficacy of GABAergic anticonvulsants, and amelioration by antagonists of secondary chloride transport.

7.
Neuroimage ; 181: 414-429, 2018 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-30025851

RESUMO

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.

8.
J Craniomaxillofac Surg ; 46(8): 1179-1184, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29884313

RESUMO

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.

9.
Sci Transl Med ; 10(443)2018 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-29848662

RESUMO

Epilepsies are characterized by recurrent seizures, which disrupt normal brain function. Alterations in neuronal excitability and excitation-inhibition balance have been shown to promote seizure generation, yet molecular determinants of such alterations remain to be identified. Pannexin channels are nonselective, large-pore channels mediating extracellular exchange of neuroactive molecules. Recent data suggest that these channels are activated under pathological conditions and regulate neuronal excitability. However, whether pannexin channels sustain or counteract chronic epilepsy in human patients remains unknown. We studied the impact of pannexin-1 channel activation in postoperative human tissue samples from patients with epilepsy displaying epileptic activity ex vivo. These samples were obtained from surgical resection of epileptogenic zones in patients suffering from lesional or drug-resistant epilepsy. We found that pannexin-1 channel activation promoted seizure generation and maintenance through adenosine triphosphate signaling via purinergic 2 receptors. Pharmacological inhibition of pannexin-1 channels with probenecid or mefloquine-two medications currently used for treating gout and malaria, respectively-blocked ictal discharges in human cortical brain tissue slices. Genetic deletion of pannexin-1 channels in mice had anticonvulsant effects when the mice were exposed to kainic acid, a model of temporal lobe epilepsy. Our data suggest a proepileptic role of pannexin-1 channels in chronic epilepsy in human patients and that pannexin-1 channel inhibition might represent an alternative therapeutic strategy for treating lesional and drug-resistant epilepsies.

10.
Epilepsy Res ; 145: 1-17, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29803953

RESUMO

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.

11.
Radiology ; 286(2): 651-658, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29023219

RESUMO

Purpose To propose and validate a modified pediatric intracerebral hemorrhage (PICH) (mPICH) score and to compare its association with functional outcome to that of the original PICH score. Materials and Methods Data from prospectively included patients were retrospectively analyzed. Consecutive patients with nontraumatic PICH who had undergone clinical follow-up were included. The study population was divided into a development cohort (2008-2012, n = 100) and a validation cohort (2013-2016, n = 43). An mPICH score was developed after variables associated with poor outcome were identified at multivariate analysis (King's Outcome Scale for Childhood Head Injury score < 5a) in the development cohort. The accuracy of the score for prediction of poor outcome was evaluated (sensitivity, specificity). Discrimination and calibration of associations between the mPICH score and poor outcome cohorts were assessed (C statistics, Hosmer-Lemeshow test). Results The mPICH score assessed as follows: brain herniation, four points; altered mental status, three points; hydrocephalus, two points; infratentorial PICH, two points; intraventricular hemorrhage, one point; PICH volume greater than 2% of total brain volume, one point. An mPICH score greater than 5 was associated with severe disability or worse, with sensitivity of 97% (95% confidence interval [CI]: 83%, 100%) and specificity of 61% (95% CI: 49%, 73%). The C statistic was 0.81 (95% CI: 0.73, 0.89). In the validation cohort, sensitivity and specificity were 95.2% (95% CI: 76%, 99%) and 77% (95% CI: 55%, 92%), respectively. There was no significant difference between the observed and predicted risks of poor outcome (P = .46). Conclusion An mPICH score was developed as a simple clinical and imaging grading scale for acute prognosis in patients with PICH. © RSNA, 2017.


Assuntos
Hemorragia Cerebral/mortalidade , Criança , Pré-Escolar , Feminino , França/epidemiologia , Humanos , Masculino , Prognóstico , Estudos Prospectivos , Curva ROC , Estudos Retrospectivos , Fatores de Risco , Índices de Gravidade do Trauma
12.
Neuro Oncol ; 20(7): 994-1002, 2018 Jun 18.
Artigo em Inglês | MEDLINE | ID: mdl-29244086

RESUMO

Background: The interval between progression and death in diffuse intrinsic pontine glioma (DIPG) is usually <6 months. However, reports of longer patient survival following radiotherapy, in the presence of radiological signs of progression, suggest that these cases may be comparable to pseudoprogression observed in adult glioblastoma. Our aim was to identify such cases and compare their multimodal MRI features with those of patients who did not present the same evolution. Methods: Multimodal MRIs of 43 children treated for DIPG were retrospectively selected at 4 timepoints: baseline, after radiotherapy, during true progression, and at the last visit. The patients were divided into 2 groups depending on whether they presented conventional MRI changes that mimicked progression. The apparent diffusion coefficient, arterial spin labeling cerebral blood flow (ASL-CBF), and dynamic susceptibility contrast perfusion relative cerebral blood volume (DSCrCBV) and flow (DSCrCBF) values were recorded for each tumor voxel, avoiding necrotic areas. Results: After radiotherapy, 19 patients (44%) showed radiological signs that mimicked progression: 16 survived >6 months following so-called pseudoprogression, with a median of 8.9 months and a maximum of 35.6 months. All 43 patients exhibited increased blood volume and flow after radiotherapy, but the 90th percentile of those with signs of pseudoprogression had a greater increase of ASL-CBF (P < 0.001). Survival between the 2 groups did not differ significantly. During true progression, DSCrCBF and DSCrCBV values increased only in patients who had not experienced pseudoprogression. Conclusions: Pseudoprogression is a frequent phenomenon in DIPG patients. This condition needs to be recognized before considering treatment discontinuation. In this study, the larger increase of the ASL-CBF ratio after radiotherapy accurately distinguished pseudoprogression from true progression.

13.
Int J Radiat Oncol Biol Phys ; 99(2): 476-485, 2017 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-28871999

RESUMO

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.


Assuntos
Neoplasias do Tronco Encefálico/diagnóstico por imagem , Neoplasias do Tronco Encefálico/radioterapia , Encéfalo/irrigação sanguínea , Glioma/diagnóstico por imagem , Glioma/radioterapia , Adolescente , Antineoplásicos/uso terapêutico , Encéfalo/efeitos da radiação , Neoplasias do Tronco Encefálico/irrigação sanguínea , Neoplasias do Tronco Encefálico/patologia , Quimioterapia Adjuvante , Criança , Pré-Escolar , Meios de Contraste , Progressão da Doença , Intervalo Livre de Doença , Cloridrato de Erlotinib/administração & dosagem , Feminino , Glioma/irrigação sanguínea , Glioma/patologia , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Estudos Prospectivos , Sirolimo/administração & dosagem , Estatísticas não Paramétricas , Análise de Sobrevida , Fatores de Tempo , Carga Tumoral/efeitos da radiação
14.
Oncotarget ; 8(32): 52543-52559, 2017 Aug 08.
Artigo em Inglês | MEDLINE | ID: mdl-28881750

RESUMO

Diffuse Instrinsic Pontine Glioma is the most aggressive form of High Grade Gliomas in children. The lack of biological material and the absence of relevant models have hampered the development of new therapeutics. Their extensive infiltration of the brainstem renders any surgical resection impossible and until recently biopsies were considered not informative enough and therefore not recommended. Thus, most models were derived from autopsy material. We aimed to develop relevant in vivo DIPG models that mimic this specific disease and its molecular diversity from tumor material obtained at diagnosis. Eight patient-derived orthotopic xenograft models were obtained after direct stereotactic injection of a mixed cell suspension containing tumor cells and stromal cells in the brainstem or thalamus of nude mice and serially passaged thereafter. In parallel, we developed 6 cell-derived xenograft models after orthotopic injection of tumor-initiating cells cultured from stereotactic biopsies. Cells were modified to express luciferase to enable longitudinal tumor growth monitoring, and fluorescent reporter proteins to trace the tumor cells in the brain. These models do not form a tumor mass, they are invasive, show the H3K27 trimethylation loss in vivo and the tumor type diversity observed in patients in terms of histone H3 mutations and lineage markers. Histological and MRI features at 11.7 Tesla show similarities with treatment naïve human DIPG, and in this respect, both direct and indirect orthotopic xenograft looked alike. These DIPG models will therefore constitute valuable tools for evaluating new therapeutic approaches in this devastating disease.

15.
Epilepsia ; 58 Suppl 1: 46-55, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-28386922

RESUMO

Hemispherectomy is a complex multistep procedure with a steep learning curve. Several surgical approaches have been developed, but each requires considerable practice to master. Four experienced pediatric neurosurgeons, who participated in the 2014 Gothenburg Pediatric Epilepsy Surgery Meeting, provided succinct technical summaries of four hemispherectomy approaches: modified functional hemispherectomy, peri-insular hemispherotomy, parasagittal hemispherotomy, and endoscopic-assisted hemispherotomy. No clinical or outcome data are included. Our intention is to reduce the slope and length of the learning curve for surgeons and to improve the understanding of the technical details of hemispherectomy surgery by nonsurgeonmembers of epilepsy teams.


Assuntos
Córtex Cerebral/cirurgia , Epilepsia/cirurgia , Hemisferectomia/métodos , Comitês Consultivos , Córtex Cerebral/diagnóstico por imagem , Craniotomia , Eletroencefalografia , Epilepsia/diagnóstico por imagem , Humanos , Processamento de Imagem Assistida por Computador , Imagem por Ressonância Magnética
16.
J Neurosurg Pediatr ; 20(1): 10-18, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28430083

RESUMO

OBJECTIVE Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome. METHODS The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995. RESULTS Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009). CONCLUSIONS Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems.


Assuntos
Hidrocefalia/cirurgia , Adolescente , Derivações do Líquido Cefalorraquidiano/efeitos adversos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Hidrocefalia/etiologia , Hidrocefalia/mortalidade , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Análise Multivariada , Falha de Prótese , Infecções Relacionadas à Prótese/mortalidade , Reoperação , Estudos Retrospectivos , Fatores de Tempo , Falha de Tratamento
17.
Neurosurgery ; 80(6): 950-956, 2017 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-28327981

RESUMO

BACKGROUND: Multiple burr hole (MBH) surgery is a simple, safe, and effective indirect technique of revascularization in moyamoya angiopathy (MM). However, it is not yet recognized as a first-line treatment. OBJECTIVE: To assess the long-term outcome and perioperative complications in a large single-center cohort of children with MM who underwent burr hole surgery. METHODS: This study is a retrospective analysis of children who underwent surgery for MM in a national reference center for pediatric stroke between 1999 and 2015. Sixty-four children (108 hemispheres, median age 7 years) were consecutively treated. The indication for revascularization was previous stroke or transient ischemic attack (TIA) or rapidly progressive disease on brain magnetic resonance imaging (MRI) and digital subtraction angiography. Children were followed with clinical examinations, telephone interviews, and MRI with any clinical recurrence of stroke or TIA used as the primary endpoint. Surgical mortality and morbidity were documented. RESULTS: Sixty-four patients were operated (bilateral MBH n = 39, unilateral procedure n = 25). At a mean follow-up of 4.2 years and 270.6 patient years, 89.1% of patients had not suffered any recurrent stroke or TIA. A second surgery was required in 5 cases after unilateral revascularization, and in 3 cases after bilateral MBH. Mortality associated with the procedure was 0. Postoperative Matsushima angiographic grading was the only predictive factor of ischemic recurrence ( P = .036). CONCLUSION: In pediatric MM, MBH compares favorably to other indirect or direct revascularization techniques in children in the prevention of stroke or TIA.


Assuntos
Revascularização Cerebral/métodos , Doença de Moyamoya/cirurgia , Crânio/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento
18.
Springerplus ; 5(1): 879, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27386327

RESUMO

BACKGROUND: Standard treatment for recurrent ependymomas is not defined. Re- irradiation has been proposed but its modalities and results are still to be explored. PATIENTS AND METHODS: From June 1994 to December 2013, 32 pediatric patients with ependymoma were re-irradiated for local (n = 15) or metastatic (n = 17) relapses. Files were reviewed retrospectively. RESULTS: Local relapses were treated with hypofractionated focal radiotherapy (hypoFFRT) (n = 8) or focal fractionated radiotherapy (FFRT) (n = 7). Metastatic relapses were treated with hypoFFRT (n = 3), FFRT (n = 3), spinal radiotherapy (n = 4) and craniospinal irradiation (CSI) (n = 7). Median PFS and OS after re-irradiation were 1.2 and 3.5 years respectively with a median follow-up of 2.1 years (0.2-11.4). For local relapses, median PFS was 2.5 years for patients treated with hypoFFRT versus 1.2 years for patients treated with FFRT (p = 0.2). For metastatic relapses, median PFS was 0.7 years for patients treated with focal radiotherapy (hypoFFRT, FFRT, spinal radiotherapy) versus 6.8 years for patients treated with CSI (p = 0.073). 15 patients achieved greater PFS after second radiotherapy (RT2) than after first radiotherapy (RT1). 27 patients (84 %) had surgery before re-irradiation. PFS was better for patients with GTR before RT2 (14.7 vs 6.7 months) (p = 0.05). 5 patients developed radionecrosis; only one required corticosteroids. CONCLUSION: Re-irradiation at relapse is a safe, feasible and potentially curative treatment. Metastatic relapse may require CSI even when isolated and re-operated. For local relapses, considering conflicting results in the literature, a randomized trial is warranted to explore fractionated focal radiotherapy versus hypofractionated focal irradiation.

19.
Radiology ; 281(2): 553-566, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27257950

RESUMO

Purpose To compare arterial spin labeling (ASL) data between low- and high-grade brain tumors in children to establish a cutoff to distinguish low- from high-grade neoplasms and to assess potential correlations between cerebral blood flow (CBF) and quantitative histologic microvascular data. Materials and Methods Approval was obtained from the regional review board. ASL data obtained in 129 children between 2011 and 2015 were retrospectively analyzed. CBF and relative CBF in the most perfused area of each neoplasm and contrast enhancement were quantified with a semiquantitative ratio. The correlation between CBF and microvascular density was analyzed in specimens stained with anti-CD34. Results were controlled in two validation cohorts with 1.5- and 3.0-T magnetic resonance (MR) imaging. Results Mean CBF was significantly higher for high-grade than for low-grade hemispheric (116 mL/min/100 g [interquartile range {IQR}, 73-131 mL/min/100 g] vs 29 mL/min/100 g [IQR, 23-35 29 mL/min/100 g], P < .001), thalamic (87 mL/min/100 g [IQR, 73-100 mL/min/100 g] vs 36 mL/min/100 g [IQR, 30-40 mL/min/100 g], P = .016), and posterior fossa (59 mL/min/100 g [IQR, 45-91 mL/min/100 g] vs 33 mL/min/100 g [IQR, 25-40 mL/min/100 g], P < .001) tumors. With a cutoff of 50 mL/min/100 g, sensitivity and specificity were 90% (95% confidence interval [CI]: 68, 100) and 93% (95% CI: 66, 100), respectively, for hemispheric tumors; 100% (95% CI: 48, 100) and 80% (95% CI: 28, 100), respectively, for thalamic tumors; and 65% (95% CI: 51, 78) and 94% (95% CI: 80, 99), respectively, for posterior fossa tumors. In posterior fossa tumors, additional use of the CBF-to-contrast enhancement ratio yielded sensitivity and specificity of 96% (95% CI: 87, 100) and 97% (95% CI: 84, 100), respectively. Use of a simple algorithm based on these values yielded an accuracy of 93% (95% CI: 87, 97). Validation sets yielded similar results, with grading accuracy of 88% (95% CI: 62, 98) with 1.5-T MR imaging and 77% (95% CI: 46, 95) with 3.0-T MR imaging. CBF was strongly correlated with microvascular density (R = 0.66, P < .001). Conclusion High-grade pediatric brain tumors display higher CBF than do low-grade tumors, and they may be accurately graded by using these values. CBF is correlated with tumor microvascular density. © RSNA, 2016 Online supplemental material is available for this article.


Assuntos
Neoplasias Encefálicas/patologia , Angiografia por Ressonância Magnética/métodos , Marcadores de Spin , Adolescente , Neoplasias Encefálicas/irrigação sanguínea , Neoplasias Encefálicas/terapia , Circulação Cerebrovascular , Criança , Pré-Escolar , Meios de Contraste , Feminino , Humanos , Lactente , Masculino , Gradação de Tumores , Estudos Retrospectivos , Sensibilidade e Especificidade
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