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PURPOSE: To develop and validate a noninvasive imaging technique for accurately assessing very slow CSF flow within shunt tubes in pediatric patients with hydrocephalus, aiming to identify obstructions that might impede CSF drainage. THEORY AND METHODS: A simulation of shunt flow enhancement of signal intensity (shunt-FENSI) signal is used to establish the relationship between signal change and flow rate. The quantification of flow enhancement of signal intensity data involves normalization, curve fitting, and calibration to match simulated data. Additionally, a phase sweep method is introduced to accommodate the impact of magnetic field inhomogeneity on the flow measurement. The method is tested in flow phantoms, healthy adults, intensive care unit patients with external ventricular drains (EVD), and shunt patients. EVDs enable shunt-flow measurements to be acquired with a ground truth measure of CSF drainage. RESULTS: The flow-rate-to-signal simulation establishes signal-flow relationships and takes into account the T1 of draining fluid. The phase sweep method accurately accounts for phase accumulation due to frequency offsets at the shunt. Results in phantom and healthy human participants reveal reliable quantification of flow rates using controlled flows and agreement with the flow simulation. EVD patients display reliable measures of flow rates. Shunt patient results demonstrate feasibility of the method and consistent flow rates for functional shunts. CONCLUSION: The results demonstrate the technique's applicability, accuracy, and potential for diagnosing and noninvasively monitoring hydrocephalus. Limitations of the current approach include a high sensitivity to motion and strict requirement of imaging slice prescription.
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Derivações do Líquido Cefalorraquidiano , Hidrocefalia , Imageamento por Ressonância Magnética , Imagens de Fantasmas , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/fisiopatologia , Imageamento por Ressonância Magnética/métodos , Adulto , Masculino , Feminino , Reprodutibilidade dos Testes , Simulação por Computador , Criança , Líquido Cefalorraquidiano/diagnóstico por imagem , Líquido Cefalorraquidiano/fisiologia , Algoritmos , Processamento de Imagem Assistida por Computador/métodosRESUMO
We sought to perform a systematic review and individual participant data meta-analysis to identify predictors of treatment response following thalamic neuromodulation in pediatric patients with medically refractory epilepsy. Electronic databases (MEDLINE, Ovid, Embase, and Cochrane) were searched, with no language or data restriction, to identify studies reporting seizure outcomes in pediatric populations following deep brain stimulation (DBS) or responsive neurostimulation (RNS) implantation in thalamic nuclei. Studies featuring individual participant data of patients with primary or secondary generalized drug-resistant epilepsy were included. Response to therapy was defined as >50% reduction in seizure frequency from baseline. Of 417 citations, 21 articles reporting on 88 participants were eligible. Mean age at implantation was 13.07 ± 3.49 years. Fifty (57%) patients underwent DBS, and 38 (43%) RNS. Sixty (68%) patients were implanted in centromedian nucleus and 23 (26%) in anterior thalamic nucleus, and five (6%) had both targets implanted. Seventy-four (84%) patients were implanted bilaterally. The median time to last follow-up was 12 months (interquartile range = 6.75-26.25). Sixty-nine percent of patients achieved response to treatment. Age, target, modality, and laterality had no significant association with response in univariate logistic regression. Until thalamic neuromodulation gains widespread approval for use in pediatric patients, data on efficacy will continue to be limited to small retrospective cohorts and case series. The inherent bias of these studies can be overcome by using individual participant data. Thalamic neuromodulation appears to be a safe and effective treatment for epilepsy. Larger, prolonged prospective, multicenter studies are warranted to further evaluate the efficacy of DBS over RNS in this patient population where resection for curative intent is not a safe option.
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Estimulação Encefálica Profunda , Epilepsia Resistente a Medicamentos , Tálamo , Humanos , Epilepsia Resistente a Medicamentos/terapia , Epilepsia Resistente a Medicamentos/fisiopatologia , Estimulação Encefálica Profunda/métodos , Criança , Tálamo/fisiopatologia , Adolescente , Resultado do Tratamento , Feminino , MasculinoRESUMO
PURPOSE: Von Hippel-Lindau (VHL) disease is an autosomal-dominantly inherited tumor predisposition syndrome. One of the most common tumors are central nervous system (CNS) hemangioblastomas. Recommendations on the initiation and continuation of the screening and surveillance program for CNS tumors in pediatric VHL patients are based on small case series and thus low evidence level. To derive more robust screening recommendations, we report on the largest monocentric pediatric cohort of VHL patients. METHODS: We performed a retrospective analysis on a pediatric cohort of 99 VHL patients consulted at our VHL center from 1992 to 2023. Clinical, surgical, genetic, and imaging data were collected and statistically analyzed. RESULTS: 42 patients (50% male) developed CNS hemangioblastomas, of whom 18 patients (56% male) underwent hemangioblastoma surgery (mean age at first surgery: 14.9 ± 1.9 years; range 10.2-17). The first asymptomatic patient was operated on at the age of 13.2 years due to tumor progress. Truncating VHL mutation carriers had a significantly higher manifestation rate (HR = 3.7, 95% CI: 1.9-7.4, p < 0.0001) and surgery rate (HR = 3.3, 95% CI: 1.2-8.9, p = 0.02) compared with missense mutation carriers. CONCLUSION: We recommend starting MRI imaging at the age of 12 years with examination intervals every (1-) 2 years depending on CNS involvement. Special attention should be paid to patients with truncating variants. Affected families should be educated regularly on potential tumor-associated symptoms to enable timely MRI imaging and eventually intervention, as CNS hemangioblastoma may develop before screening begins. GERMAN CLINICAL TRIALS REGISTER REGISTRATION NUMBER: DRKS00029553, date of registration 08/16/2022, retrospectively registered.
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Hemangioblastoma , Doença de von Hippel-Lindau , Humanos , Doença de von Hippel-Lindau/genética , Doença de von Hippel-Lindau/complicações , Hemangioblastoma/cirurgia , Hemangioblastoma/genética , Hemangioblastoma/patologia , Masculino , Feminino , Adolescente , Criança , Estudos Retrospectivos , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/cirurgia , Neoplasias Cerebelares/patologia , Neoplasias do Sistema Nervoso Central/genética , Neoplasias do Sistema Nervoso Central/diagnóstico , Neoplasias do Sistema Nervoso Central/cirurgia , Neoplasias do Sistema Nervoso Central/patologia , Seguimentos , Proteína Supressora de Tumor Von Hippel-Lindau/genéticaRESUMO
Pediatric epilepsy has a worldwide prevalence of approximately 1% (Berg et al., Handb Clin Neurol 111:391-398, 2013) and is associated with not only lower quality of life but also long-term deficits in executive function, significant psychosocial stressors, poor cognitive outcomes, and developmental delays (Schraegle and Titus, Epilepsy Behav 62:20-26, 2016; Puka and Smith, Epilepsia 56:873-881, 2015). With approximately one-third of patients resistant to medical control, surgical intervention can offer a cure or palliation to decrease the disease burden and improve neurological development. Despite its potential, epilepsy surgery is drastically underutilized. Even today only 1% of the millions of epilepsy patients are referred annually for neurosurgical evaluation, and the average delay between diagnosis of Drug Resistant Epilepsy (DRE) and surgical intervention is approximately 20 years in adults and 5 years in children (Solli et al., Epilepsia 61:1352-1364, 2020). It is still estimated that only one-third of surgical candidates undergo operative intervention (Pestana Knight et al., Epilepsia 56:375, 2015). In contrast to the stable to declining rates of adult epilepsy surgery (Englot et al., Neurology 78:1200-1206, 2012; Neligan et al., Epilepsia 54:e62-e65, 2013), rates of pediatric surgery are rising (Pestana Knight et al., Epilepsia 56:375, 2015). Innovations in surgical approaches to epilepsy not only minimize potential complications but also expand the definition of a surgical candidate. In this chapter, three alternatives to classical resection are presented. First, laser ablation provides a minimally invasive approach to focal lesions. Next, both central and peripheral nervous system stimulation can interrupt seizure networks without creating permanent lesions. Lastly, focused ultrasound is discussed as a potential new avenue not only for ablation but also modulation of small, deep foci within seizure networks. A better understanding of the potential surgical options can guide patients and providers to explore all treatment avenues.
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Epilepsia , Procedimentos Neurocirúrgicos , Criança , Humanos , Epilepsia Resistente a Medicamentos/cirurgia , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia/cirurgia , Terapia a Laser/métodos , Procedimentos Neurocirúrgicos/métodosRESUMO
Favorable clinical outcomes in adult and pediatric neurosurgical oncology generally depend on the extent of tumor resection (EOR). Maximum safe resection remains the main aim of surgery in most intracranial tumors. Despite the accuracy of intraoperative magnetic resonance imaging (iMRI) in the detection of residual intraoperatively, it is not widely implemented worldwide owing to enormous cost and technical difficulties. Over the past years, intraoperative ultrasound (IOUS) has imposed itself as a valuable and reliable intraoperative tool guiding neurosurgeons to achieve gross total resection (GTR) of intracranial tumors.Being less expensive, feasible, doesn't need a high level of training, doesn't need a special workspace, and being real time with outstanding temporal and spatial resolution; all the aforementioned advantages give a superiority for IOUS in comparison to iMRI during resection of brain tumors.In this chapter, we spot the light on the technical nuances, advanced techniques, outcomes of resection, pearls, and pitfalls of the use of IOUS during the resection of brain tumors.
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Neoplasias Encefálicas , Hemisferectomia , Psicocirurgia , Adulto , Criança , Humanos , Ultrassonografia , Neoplasias Encefálicas/diagnóstico por imagem , Encéfalo/diagnóstico por imagemRESUMO
BACKGROUND: Remifentanil, an ultra-short-acting µ-opioid receptor agonist, is commonly used for anesthetic management due to excellent adjustability. Remifentanil is known to cause sinus bradycardia, however, because it has a direct negative chronotropic effect on the cardiac conduction system and there is an indirect negative chronotropic effect via the parasympathetic nervous system. CASE PRESENTATION: An 8-year-old Japanese boy was diagnosed with acute hydrocephalus due to a brain tumor in the fourth ventricle and underwent emergency surgery. Imaging examination showed brainstem compression. Endoscopic third ventriculostomy and ventriculoperitoneal shunt surgery were scheduled. Remifentanil was started during induction of general anesthesia, but electrocardiogram showed sinus bradycardia, then Wenckebach-type atrioventricular block, and then complete atrioventricular block. Remifentanil was immediately discontinued, and we administered atropine sulfate. Complete atrioventricular block was restored to sinus rhythm. When remifentanil was restarted, however, the electrocardiogram again showed sinus bradycardia, Wenckebach-type atrioventricular block, and then complete atrioventricular block. Remifentanil was again immediately discontinued, we administered adrenaline, and then complete atrioventricular block was restored to sinus rhythm. Fentanyl was used instead of remifentanil with continuous infusion of dopamine. There has since been no further occurrence of complete atrioventricular block. CONCLUSIONS: This is the first known case of complete atrioventricular block in a pediatric patient with increased intracranial pressure seemingly caused by administration of remifentanil.
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Bloqueio Atrioventricular , Hidrocefalia , Remifentanil , Humanos , Masculino , Remifentanil/administração & dosagem , Remifentanil/efeitos adversos , Criança , Bloqueio Atrioventricular/induzido quimicamente , Hidrocefalia/cirurgia , Neoplasias Encefálicas/cirurgia , Anestesia Geral/métodos , Anestesia Geral/efeitos adversos , Piperidinas/efeitos adversos , Piperidinas/administração & dosagem , Analgésicos Opioides/efeitos adversos , Analgésicos Opioides/administração & dosagem , Anestésicos Intravenosos/efeitos adversos , Anestésicos Intravenosos/administração & dosagemRESUMO
INTRODUCTION: Pediatric Neurosurgery as a subspeciality started to emerge during the late 1950s, with only a few dedicated pediatric neurosurgeons in the Western world. Over the last few decades, the awareness that children require subspecialized care by dedicated pediatric neurosurgeons and an interdisciplinary team has been growing worldwide, leading to an increase in pediatric neurosurgeons. Several studies have shown that subspecialized care for pediatric patients improves outcomes and is cost-effective. This survey aims to assess the current setting of pediatric neurosurgery and training of neurosurgical residents in pediatric neurosurgery in Switzerland. METHODS: We conducted an online survey by sending e-mail invitations in 2021 to all neurosurgical residents in Switzerland. The survey included questions regarding the participants' demographics, current workplace structures, the care of specific pediatric neurosurgical pathologies, and participants' opinions of the Swiss training program for pediatric neurosurgery and possible improvement. We defined at the beginning of the survey that a pediatric neurosurgeon is a board-certified neurosurgeon with at least one year of dedicated pediatric neurosurgical fellowship training abroad. RESULTS: We received a total of 25 responses from residents, of which 20 (80%) were male. Twenty-two participants (88%) worked in one of seven major hospitals in Switzerland at the time of the survey, and four (16%) were interested in pursuing a fellowship in pediatric neurosurgery. Seven (35%) and five residents (25%) feel comfortable taking care on the ward of a craniosynostosis and hydrocephalus patient younger than 6 months, respectively. Twelve residents (60%) feel comfortable taking care of a pediatric brain tumor patient. The majority (n = 22, 88%) of all residents agree that a fellowship-trained pediatric neurosurgeon should treat children, while two (8%) residents state that any neurosurgeon with an interest in pediatric neurosurgery should be able to treat children. All residents (n = 25, 100%) agree that pediatric neurosurgery training and care in Switzerland needs to be improved. CONCLUSION: Pediatric neurosurgery training in Switzerland is rather heterogeneous and not very well structured, with varying frequencies of children-specific neurosurgical pathologies. Most residents agreed that a subspecialized pediatric neurosurgeon should oversee the care of children in neurosurgery, while all agree that pediatric neurosurgical training and care should be improved in Switzerland.
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Internato e Residência , Neurocirurgia , Pediatria , Humanos , Suíça , Neurocirurgia/educação , Pediatria/educação , Masculino , Feminino , Inquéritos e Questionários , Neurocirurgiões/educação , Adulto , Procedimentos Neurocirúrgicos/educaçãoRESUMO
PURPOSE: Geographic access to physicians has been shown to be unevenly distributed in the USA, with those in closer proximity having superior outcomes. The purpose of this study was to describe how geographic access to pediatric neurosurgeons varies across socioeconomic and demographic factors. METHODS: Actively practicing neurosurgeons were identified by matching several registries and membership logs. This data was used to find their primary practice locations and the distance the average person in a county must travel to visit a surgeon. Counties were categorized into "surgeon deserts" and "surgeon clusters," which were counties where providers were significantly further or closer to its residents, respectively, compared to the national average. These groups were also compared for differences in population characteristics using data obtained from the 2020 American Community Survey. RESULTS: A total of 439 pediatric neurosurgeons were identified. The average person in a surgeon desert and cluster was found to be 189.2 ± 78.1 miles and 39.7 ± 19.6 miles away from the nearest pediatric neurosurgeon, respectively. Multivariate analyses showed that higher Rural-Urban Continuum (RUC) codes (p < 0.001), and higher percentages of American Indian (p < 0.001) and Hispanic (p < 0.001) residents were independently associated with counties where the average person traveled significantly further to surgeons. CONCLUSION: Patients residing in counties with greater RUC codes and higher percentages of American Indian and Hispanic residents on average need to travel significantly greater distances to access pediatric neurosurgeons.
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Neurocirurgiões , Cirurgiões , Humanos , Criança , Estados Unidos , Fatores Sociodemográficos , Análise Multivariada , Sistema de RegistrosRESUMO
PURPOSE: Cerebrospinal fluid (CSF) leakage is a challenging complication of intradural cranial surgery, and children are particularly at risk. The use of dural sealants confers protection in adults, but pediatric studies are scarce. We evaluated the safety and efficacy of Evicel® fibrin sealant as an adjunct to primary dural suturing in children undergoing cranial surgery. METHODS: A multicenter trial prospectively enrolled pediatric subjects (< 18 years) undergoing cranial neurosurgery who, upon completion of primary sutured dural repair, experienced CSF leakage. As agreed by the EMA Evicel® Pediatric Investigation Plan, 40 subjects were intra-operatively randomized 2:1 to Evicel® or additional sutures ('Sutures'). Data analysis was descriptive. The efficacy endpoint was treatment success rate, with success defined as intra-operative watertight closure after provocative Valsalva maneuver (primary endpoint). Safety endpoints were postoperative CSF leakage (incisional CSF leakage, pseudomeningocele or both) and surgical site complications (secondary endpoints). RESULTS: Forty subjects (0.6-17 years) were randomized to Evicel® (N = 25) or Sutures (N = 15) (intention-to-treat). Intracranial tumor was the most common indication and procedures were mostly supratentorial craniotomies. Success rates were 92.0% for Evicel® and 33.3% for Sutures, with a 2.76 estimated ratio of success rates (Farrington-Manning 95% CI [1.53, 6.16]). Sensitivity analyses in per-protocol and safety sets showed similar results. Despite a higher rescue treatment rate, the frequencies of postoperative CSF leakage and wound complications were higher for Sutures than for Evicel®. CONCLUSION: This small-scale prospective study shows Evicel® treatment to be safe and effective as an adjunct to primary sutured dura mater closure in a pediatric population. Compared to additional sutures, Evicel® was associated with reduced postoperative CSF leakage and surgical site complications. (Trial registration: The trial was registered as NCT02309645 and EudraCT 2013-003558-26).
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Vazamento de Líquido Cefalorraquidiano , Dura-Máter , Adesivo Tecidual de Fibrina , Procedimentos Neurocirúrgicos , Humanos , Adesivo Tecidual de Fibrina/uso terapêutico , Feminino , Masculino , Criança , Pré-Escolar , Adolescente , Lactente , Procedimentos Neurocirúrgicos/métodos , Dura-Máter/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Resultado do Tratamento , Adesivos Teciduais/uso terapêutico , Estudos Prospectivos , Técnicas de SuturaRESUMO
PURPOSE: Calvarial dermoid and epidermoid cysts are benign lesions common in pediatric neurosurgery. Diagnosis is primarily clinical, with frequent but inconsistent use of imaging. Dermoids have been shown to possess distinct sonographic features, but ultrasound (US) remains underutilized in their management. The purpose of this study is to investigate the independent reliability of US in managing pediatric calvarial dermoids and distinguishing them from other calvarial lesions. METHODS: A retrospective review of consecutive patients ≤ 21 years of age with surgically resected calvarial masses between 2017-2024 was performed. Demographic, clinical, and imaging data were analyzed. Pearson chi-squared tests were used for comparison of categorical variables and a binomial linear model was generated controlling for age, lesion tenderness, growth, and suture location. RESULTS: Fifty-nine patients with 61 lesions (31 in females; median age 13 months) were included. Dermoids were more common in younger patients (median age 12 months), along suture lines, and were less likely to present with tenderness (p < 0.001) or rapid growth (p = 0.003). Ultrasound was used in 83% of cases and was the sole imaging modality in 33%. On multivariate analysis, suture location was a significant positive predictor of a dermoid diagnosis (OR = 8.08, 95% CI = 1.67-44.18), while rapid growth was a significant negative predictor (OR = 0.08, 95% CI = 0.003-0.80). CONCLUSION: Ultrasound presents a sensitive and reliable method for the evaluation of most pediatric calvarial lesions, especially dermoid cysts, and warrants being part of standard workup. With appropriate patient selection, US obviates the need for additional imaging in pediatric patients.
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BACKGROUND: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes. MATERIAL AND METHODS: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up. RESULTS: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%. CONCLUSIONS: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis.
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Abscesso Encefálico , COVID-19 , Empiema Subdural , Otite , Sinusite , Criança , Humanos , Pandemias , COVID-19/complicações , Abscesso Encefálico/epidemiologia , Empiema Subdural/etiologia , Sinusite/complicações , Otite/complicações , Otite/epidemiologia , Estudos RetrospectivosRESUMO
PURPOSE: The management of depressed skull fractures in infants can be either conservative or surgical. This study aimed to examine the outcomes of management with a negative-pressure vacuum device on depressed skull fractures in newborns. METHODS: Twenty-eight patients (aged 1-6 days) with simple depressed skull fractures underwent skull elevation using negative-pressure vacuum devices. A protocol for nonsurgical management was adopted for infants with such fractures between 2010 and 2023. All patients were initially evaluated with neurological examination and complementary assessments-hematological and coagulation studies, transfontanel transcranial ultrasound, skull radiography, and computed tomography scanning with three-dimensional reconstruction-according to availability and clinical needs. Gentle (negative) extraction pressure was applied with one of several devices (according to institutional availability) for a maximum duration of 60 s; this was performed as soon as possible after diagnosis, preferably within 72 h. Follow-up data, available in the clinical records, were reported. RESULTS: All patients exhibited satisfactory elevation of the depressed bone without associated injuries, except one patient who presented with an associated cephalohematoma which prevented optimal device coupling to generate sufficient vacuum pressure for correction. Neither neurological deficits nor development of epilepsy was noted; normal neurological assessment and oral alimentation tolerance were confirmed within 24 h post procedure. CONCLUSIONS: According to our data, ping-pong skull fracture elevation using the vacuum method is a safe and satisfactory treatment in the neonatal period. Early treatment allows for quick resolution, and in our opinion is the strategy of choice for depressed skull fractures in newborns.
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Fratura do Crânio com Afundamento , Fraturas Cranianas , Lactente , Humanos , Recém-Nascido , Vácuo , Crânio , CabeçaRESUMO
PURPOSE: To describe a surgical technique for posterior cerebral revascularization in pediatric patients with moyamoya arteriopathy. Here, we describe the clinical characteristics, surgical indications, operative techniques, and clinical and radiographic outcomes in a series of pediatric patients with moyamoya disease affecting the posterior cerebral artery (PCA) territory. METHODS: A retrospective single-center series of all pediatric patients with moyamoya disease who presented to our institute between July 2009 through August 2019 were reviewed. The clinical characteristics, surgical indications, operative techniques, and long-term clinical and radiographic outcomes of pediatric moyamoya patients with PCA territory ischemia were collected and analyzed. RESULTS: A total of 10 PCA revascularization procedures were performed in 9 patients, 5 female, ages 1 to 11.1 years (average 5.2 years). Complications included 1 stroke, with no infections, hemorrhages, seizures, or deaths. One patient had less than 1 year of radiographic and clinical follow-up. In 8 of 9 patients with at least 1 year of radiographic follow-up, there was engraftment of surgical vessels present in all cases. No new strokes were identified on long-term follow-up despite the radiographic progression of the disease. In the 8 cases available for analysis, the average follow-up was 50.8 months with a range of 12 to 117 months. CONCLUSIONS: PCA territory ischemia in patients with progressive moyamoya disease can be surgically treated with indirect revascularization. Here, we describe our experience with PCA revascularization procedures for moyamoya disease, including pial pericranial dural (PiPeD) revascularization and pial synangiosis utilizing the occipital artery. These surgical options may be useful for decreasing the risk of stroke in pediatric moyamoya patients with severe posterior circulation disease.
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Isquemia Encefálica , Revascularização Cerebral , Doença de Moyamoya , Acidente Vascular Cerebral , Criança , Humanos , Feminino , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Angiografia Cerebral , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/etiologia , Isquemia Encefálica/cirurgia , Infarto Cerebral/etiologia , Acidente Vascular Cerebral/etiologia , Revascularização Cerebral/métodosRESUMO
PURPOSE: Cerebral sinovenous thrombosis is an increasingly recognized cause of stroke in children and neonates. Its true incidence appears to be underestimated. Despite being a rare event, certain studies have found a correlation between subdural hemorrhage and cerebral sinus thrombosis. The literature suggests that spontaneous cerebral sinovenous thrombosis in the pediatric population may lead to the occurrence of a subdural hemorrhage. In this report, we present a case of cerebral venous thrombosis associated with chronic subdural hematoma and review the literature to highlight the importance of these conditions. CASE REPORT: An 11-year-old boy was admitted in the neurosurgery department with headache and a neurological examination without changes. The imaging studies identified a heterogeneous subdural collection in the fronto-temporo-parietal region. The patient underwent surgical drainage of the subdural hematoma, and the procedure was performed without complications. The magnetic resonance and angiography showed an extensive thrombosis of the superior sagittal sinus, extending downward to the occipital sinus and partially to the right transverse sinus. CONCLUSIONS: Appropriate management in the diagnosis and an early treatment of dural sinus thrombosis associated with subdural hemorrhage can reduce the risk of recurrence and improve the clinical outcome.
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Cavidades Cranianas , Trombose dos Seios Intracranianos , Criança , Masculino , Recém-Nascido , Humanos , Hematoma Subdural/complicações , Hematoma Subdural/diagnóstico por imagem , Hematoma Subdural/cirurgia , Trombose dos Seios Intracranianos/complicações , Trombose dos Seios Intracranianos/diagnóstico por imagem , Trombose dos Seios Intracranianos/cirurgia , Imageamento por Ressonância Magnética/efeitos adversos , Seio Sagital Superior/patologiaRESUMO
Sporadic vestibular schwannomas (VSs) are rare in children. When occurred in the pediatric population, they usually appear bilaterally and are related to neurofibromatosis type 2 (NF2). The current study reports a 4-year-old boy without family history of VS or NF2 who presented with a large (5.7-cm) VS involving the right cerebellopontine angle and internal auditory canal. Through seven-staged surgical interventions and two stereotactic γknife radiosurgery, the disease was stabilized. At 2-year follow-up, the child had right ear hearing loss, grade IV facial palsy, and normal motor function and gait. No definite evidence of gene mutation regarding NF2 can be identified after sequence analysis and deletion/duplication testing. This case highlights the significance of considering the possibility of sporadic VSs, even in very young children. It emphasizes the importance of not overlooking initial symptoms, as they may indicate the presence of a large tumor and could potentially result in delayed diagnosis.
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Neuroma Acústico , Humanos , Masculino , Pré-Escolar , Neuroma Acústico/cirurgia , Neuroma Acústico/diagnóstico por imagem , RadiocirurgiaRESUMO
Craniosynostosis refers to the premature fusion of one or more of the fibrous cranial sutures connecting the bones of the skull. Machine learning (ML) is an emerging technology and its application to craniosynostosis detection and management is underexplored. This systematic review aims to evaluate the application of ML techniques in the diagnosis, severity assessment, and predictive modeling of craniosynostosis. A comprehensive search was conducted on the PubMed and Google Scholar databases using predefined keywords related to craniosynostosis and ML. Inclusion criteria encompassed peer-reviewed studies in English that investigated ML algorithms in craniosynostosis diagnosis, severity assessment, or treatment outcome prediction. Three independent reviewers screened the search results, performed full-text assessments, and extracted data from selected studies using a standardized form. Thirteen studies met the inclusion criteria and were included in the review. Of the thirteen papers examined on the application of ML to the identification and treatment of craniosynostosis, two papers were dedicated to sagittal craniosynostosis, five papers utilized several different types of craniosynostosis in the training and testing of their ML models, and six papers were dedicated to metopic craniosynostosis. ML models demonstrated high accuracy in identifying different types of craniosynostosis and objectively quantifying severity using innovative metrics such as metopic severity score and cranial morphology deviation. The findings highlight the significant strides made in utilizing ML techniques for craniosynostosis diagnosis, severity assessment, and predictive modeling. Predictive modeling of treatment outcomes following surgical interventions showed promising results, aiding in personalized treatment strategies. Despite methodological diversities among studies, the collective evidence underscores ML's transformative potential in revolutionizing craniosynostosis management.
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Craniossinostoses , Aprendizado de Máquina , Craniossinostoses/cirurgia , Craniossinostoses/diagnóstico , HumanosRESUMO
PURPOSE: This study aims to provide an exhaustive analysis of pediatric low-grade gliomas (pLGGs) in the cerebellar hemispheres, focusing on incidence, clinical characteristics, surgical outcomes, and prognosis. It seeks to enhance understanding and management of pLGGs in the pediatric population. METHODS: We conducted an observational, descriptive, retrospective, and cross-sectional study at a pediatric hospital, reviewing medical records of 30 patients with cerebellar hemispheric pLGGs treated from December 2014 to January 2023. Data collection included demographics, clinical presentation, imaging findings, surgical approach, postoperative complications, histopathological diagnosis, hydrocephalus management, and follow-up. Molecular markers and adjuvant therapies were also analyzed. RESULTS: The cohort predominantly presented with cerebellar symptoms, with 60% showing hydrocephalus at diagnosis. MRI with gadolinium was crucial for diagnosis. Surgical focus was on achieving gross total resection (GTR), accomplished in 70% of cases. Postsurgical hydrocephalus was less common, and cerebellar mutism was not reported. While a complete molecular analysis was not performed in all cases, available data suggest significant influence of molecular markers on prognosis and therapeutic options of pLGGs. CONCLUSIONS: This study highlights the unique clinical and molecular characteristics of cerebellar hemispheric pLGGs in children. The lower incidence of postoperative hydrocephalus and absence of cerebellar mutism are notable findings. Emphasizing a multidisciplinary approach, our findings contribute to a deeper understanding of pediatric pLGGs, underscoring the need for personalized treatment strategies and vigilant follow-up.
Assuntos
Neoplasias Cerebelares , Glioma , Humanos , Feminino , Masculino , Criança , Glioma/cirurgia , Glioma/terapia , Glioma/diagnóstico , Neoplasias Cerebelares/cirurgia , Neoplasias Cerebelares/terapia , Pré-Escolar , Estudos Retrospectivos , Adolescente , Estudos Transversais , Lactente , Hospitais Pediátricos , Centros de Atenção Terciária , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Procedimentos Neurocirúrgicos/métodosRESUMO
Incidental brain tumor findings in children involve the unexpected discovery of brain lesions during imaging for unrelated reasons. These findings differ significantly from those in adults, requiring a focus on pediatric-specific approaches in neurosurgery, neuroimaging, and neuro-oncology. Understanding the prevalence, progression, and management of these incidentalomas is crucial for informed decision-making, balancing patient welfare with the risks and benefits of intervention. Incidental brain tumors are observed in about 0.04-5.7% of cases, with most suspected low-grade lesions in children showing a benign course, though up to 3% may undergo malignant transformation. Treatment decisions are influenced by factors such as patient age, tumor characteristics, and family anxiety, with conservative management through surveillance often preferred. However, upfront surgery may be considered in cases with low surgical risk. Initial follow-up typically involves a comprehensive MRI after three months, with subsequent scans spaced out if the lesion remains stable. Changes in imaging or symptoms during follow-up could indicate malignant transformation, prompting consideration of surgery or biopsy. Several challenges and controversies persist, including the role of upfront biopsy for molecular profiling, the use of advanced imaging techniques like PET-CT and magnetic resonance spectroscopy, and the implications of the child's age at diagnosis. These issues highlight the need for further research to guide management and improve outcomes in pediatric patients with incidental brain tumor findings.
RESUMO
BACKGROUND: Cavernous malformations (CMs), also known as cavernomas or cavernous angiomas, are vascular malformations characterized by sinusoidal spaces lined by endothelial cells. Giant CMs (GCMs) are extremely rare, with limited understanding of their presentation and management. We present a case of symptomatic GCM in a newborn and review the literature on this rare entity. CASE DESCRIPTION: A 1-month-old newborn presented with focal seizures and signs of increased intracranial pressure. Imaging revealed a massive right frontal-parietal GCM, prompting surgical resection. Histopathological examination confirmed the diagnosis of cerebral cavernous malformation. The patient recovered well postoperatively with no neurological deficits. CONCLUSIONS: GCMs are exceedingly rare in children and have not been reported in newborns until now. Symptoms typically include seizures and mass effects. Gross total resection is the standard treatment, offering favorable outcomes. Further research is needed to understand the natural history and optimal management of GCMs, particularly in newborns, emphasizing the importance of heightened clinical awareness for timely diagnosis and appropriate management.
Assuntos
Hemangioma Cavernoso do Sistema Nervoso Central , Feminino , Humanos , Masculino , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico por imagem , Hemangioma Cavernoso do Sistema Nervoso Central/patologia , Imageamento por Ressonância Magnética , LactenteRESUMO
The use of endoscopic third ventriculostomy (ETV) for treatment of pediatric hydrocephalus has higher failure rates in younger patients. Here we investigate the impact of select perioperative variables, specifically gestational age, chronological age, birth weight, and surgical weight, on ETV failure rates. A retrospective review was performed on patients treated with ETV - with or without choroid plexus cauterization (CPC) - from 2010 to 2021 at a large academic center. Analyses included Cox regression for independent predictors and Kaplan-Meier survival curves for time to-event outcomes. In total, 47 patients were treated with ETV; of these, 31 received adjunctive CPC. Overall, 66% of the cohort experienced ETV failure with a median failure of 36 days postoperatively. Patients aged < 6 months at time of surgery experienced 80% failure rate, and those > 6 months at time of surgery experienced a 41% failure rate. Univariate Cox regression analysis showed weight at the time of ETV surgery was significantly inversely associated with ETV failure with a hazard ratio of 0.92 (95% CI 0.82, 0.99). Multivariate analysis redemonstrated the inverse association of weight at time of surgery with ETV failure with hazard ratio of 0.76 (95% CI 0.60, 0.92), and sensitivity analysis showed < 4.9 kg as the optimal cutoff predicting ETV/CPC failure. Neither chronologic age nor gestational age were found to be significantly associated with ETV failure.In this study, younger patients experienced higher ETV failure rates, but multivariate regression found that weight was a more robust predictor of ETV failure than chronologic age or gestational age, with an optimal cutoff of 4.9 kg in our small cohort. Given the limited sample size, further study is needed to elucidate the independent role of weight as a peri-operative variable in determining ETV candidacy in young infants. Previous presentations: Poster Presentation, Congress of Neurological Surgeons.