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OBJECTIVES: To report pediatric intensivists' and pediatric neurosurgeons' responses to case-based scenarios about plasma and platelet transfusions before intracranial pressure (ICP) monitor placement in children with severe traumatic brain injury (TBI). DESIGN: Cross-sectional, electronic survey to evaluate reported plasma and platelet transfusion decisions in eight scenarios of TBI in which ICP monitor placement was indicated. SETTING: Survey administered through the Pediatric Acute Lung Injury and Sepsis Investigators and the American Association of Neurologic Surgeons. SUBJECTS: Pediatric intensivists and pediatric neurosurgeons. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 184 participants responded (85 identified as pediatric intensivists and 54 as pediatric neurosurgeons). In all eight scenarios, the majority of respondents reported that they would base their decision-making about plasma transfusion on international normalized ratio (INR) alone (60-69%), or platelet transfusion on platelet count alone (83-86%). Pediatric intensivists, as opposed to pediatric neurosurgeons, more frequently reported that they would have used viscoelastic testing in their consideration of plasma transfusion (32% vs. 7%, p < 0.001), as well as to guide platelet transfusions (29 vs. 8%, p < 0.001), for the case-based scenarios. For all relevant case-based scenarios, pediatric neurosurgeons in comparison with pediatric reported that they would use a lower median (interquartile range [IQR]) INR threshold for plasma transfusion (1.5 [IQR 1.4-1.7] vs. 2.0 [IQR 1.5-2.0], p < 0.001). Overall, in all respondents, the reported median platelet count threshold for platelet transfusion in the case-based scenario was 100 (IQR 50-100) ×10 9 /L, with no difference between specialties. CONCLUSIONS: Despite little evidence showing efficacy, when we tested specialists' decision-making, we found that they reported using INR and platelet count in pediatric case-based scenarios of TBI undergoing ICP monitor placement. We also found that pediatric intensivists and pediatric neurosurgeons had differences in decision-making about the scenarios.
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Lesões Encefálicas Traumáticas , Transfusão de Plaquetas , Humanos , Criança , Neurocirurgiões , Estudos Transversais , Transfusão de Componentes Sanguíneos , Plasma , Lesões Encefálicas Traumáticas/terapiaRESUMO
PURPOSE: Access to the pineal region has always represented a fascinating challenge to the neurosurgeons. Almost equally thrilling is the historical evolution from the hard beginnings with extremely high mortality rates to the current surgical outcomes, with excellent resection rates without long-term morbidity for most of the patients. The purpose of this paper is to provide an overview of the historical evolution of the occipital interhemispheric transtentorial (OITT) approach and its role in the development of access to the pineal region. METHODS: Review of the literature highlights the occipital transtentorial approach from the historical context prior its description and the beginning to the current modifications and new recent insights. RESULTS: The occipital transtentorial approach described initially by Poppen in 1966 has played a key role in the progress and success accessing the pineal area. CONCLUSION: This historical review aims to highlight the extraordinary effort of those neurosurgeons who guided and led the development of one of the most important approaches to the pineal region.
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Procedimentos Neurocirúrgicos , Glândula Pineal , Humanos , História do Século XX , Procedimentos Neurocirúrgicos/história , Procedimentos Neurocirúrgicos/métodos , Glândula Pineal/cirurgia , História do Século XXI , História do Século XIX , Lobo Occipital/cirurgiaRESUMO
INTRODUCTION: Outcomes for pineal region and superior cerebellar tumors in young children often hinge on extent of microsurgical resection, and thus choosing an approach that provides adequate visualization of pathology is essential. The occipital interhemispheric transtentorial (OITT) approach provides excellent exposure while minimizing cerebellar retraction. However, this approach has not been widely accepted as a viable option for very young children due to concerns for potential blood loss when incising the tentorium. The aim of this paper is to characterize our recent institutional experience with the occipital interhemispheric transtentorial approach (OITT) for tumor resection in infants and toddlers. METHODS: A retrospective study was performed between 2016 and 2023 of pediatric patients less than 36 months of age who underwent OITT for tumor resection at a high-volume referral center. Patients with at least 3 months of postoperative follow-up and postoperative MRI were included. Primary outcomes included extent of resection, intraoperative and postoperative complications, and neurologic outcome. Secondary outcomes included length of stay and estimated blood loss. RESULTS: Eight patients, five male, were included. The median age at the time of surgery was 10 months (range 5-36 months). Presenting symptoms included macrocephaly, nausea/vomiting, strabismus, gait instability, or milestone regression. Hydrocephalus was present preoperatively in all patients. Average tumor volume was 38.6 cm3, ranging from 1.3 to 71.9 cm3. All patients underwent an OITT approach for tumor resection with stereotactic guidance. No intraoperative complications occurred, and no permanent neurologic deficits developed postoperatively. Gross total resection was achieved in all cases per postoperative MRI report, and no instances of new cerebellar, brainstem, or occipital lobe ischemia were noted. CONCLUSIONS: OITT approach for tumor resection in very young children (≤ 36 months) is an effective strategy with an acceptable safety profile. In our series, no significant intraoperative or postoperative complications occurred. To our knowledge, this is the first report describing this technique specifically in patients less than 36 months of age.
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Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias , Humanos , Lactente , Masculino , Feminino , Estudos Retrospectivos , Pré-Escolar , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/etiologia , Resultado do Tratamento , Neoplasias Cerebelares/cirurgia , Neoplasias Encefálicas/cirurgia , Pinealoma/cirurgiaRESUMO
INTRODUCTION: Diffuse midline glioma (DMG) of the pons occurs in pediatric patients and carries a dismal prognosis. Biopsy is not necessary for diagnosis but provides information, particularly H3K27M status, with prognostic implications. Additionally, biopsy information may open therapeutic options such as clinical trials that require mutation status. Therefore, we sought to assess the safety of surgical biopsy in DMG patients as well as its potential impact on clinical course. METHODS: Retrospective analysis of patients who were radiographically and clinically diagnosed with pontine DMG in the last 5 years was performed. We assessed demographic, clinical, radiographic, surgical, and follow-up data. RESULTS: 25 patients were included; 18 (72%) underwent biopsy while 7 (28%) declined. 12 biopsies (67%) were performed with robotic arm and 5 (27%) with frameless stereotaxy. Three biopsied patients (17%) experienced new post-operative neurologic deficits (1 facial palsy, 1 VI nerve palsy and 1 ataxia) that all resolved at 2-week follow-up. All biopsies yielded diagnostic tissue. Fourteen patients (78%) had H3K27M mutation. Median OS for H3K27M patients was 10 months compared to 11 months in the wild-type patients (p = 0.30, log-rank test). Median OS for patients enrolled in clinical trials was 12 months compared to 8 months for non-trial patients (p = 0.076). CONCLUSION: In our series, stereotactic pontine DMG biopsies did not carry any permanent deficit or complication and yielded diagnostic tissue in all patients. Similar post-operative course was observed in both robot-assisted and frameless stereotactic approaches. There was no significant difference in survival based on mutation status or clinical trial enrollment.
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Neoplasias Encefálicas , Glioma , Criança , Humanos , Biópsia , Neoplasias Encefálicas/patologia , Glioma/genética , Glioma/cirurgia , Glioma/diagnóstico , Mutação , Ponte/patologia , Ponte/cirurgia , Estudos RetrospectivosRESUMO
BACKGROUND: The subtonsillar transcerebellomedullary approach (STA) has been well established as one of the surgical options to access lesions in the foramen of Luschka. The middle cerebellar peduncle has been defined as the superior limit of this corridor, and tumors extending beyond this landmark were often approached through combined accesses. METHOD: We illustrate the access to the suprasellar cistern, from a purely STA for a large tumor arising from the foramen of Luschka. CONCLUSION: This manuscript demonstrates the STA as a valid alternative for certain tumors of the foramen of Luschka, which extend into crural and suprasellar cisterns.
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Procedimentos Neurocirúrgicos , HumanosRESUMO
INTRODUCTION: The posterior fossa is the most common intracranial location for pediatric ependymoma. While ependymoma usually arises from the ventricular lining of the fourth ventricle as a solid mass, it rarely originates from the brainstem. Grade II ependymomas also infrequently appear as a cavitary ring-enhancing lesion. CASE PRESENTATION: We describe a case of a 6-year-old boy with an ependymoma arising within the medulla with imaging features of a thick-walled rim-enhancing cavitary lesion. A stereotactic biopsy was obtained which confirmed a grade II ependymoma. The patient received focal proton beam radiation therapy and is doing well with no concerns for disease progression at 28 months after diagnosis. CONCLUSION: Posterior fossa ependymomas typically arise from ependymal cells within the fourth ventricle or foramina of Luschka. They rarely invade or arise within the brainstem parenchyma. Our case had atypical imaging findings in addition to the atypical tumor location. The lesion was described as a thick-walled rim-enhancing focal cystic necrotic lesion centered within the medulla with surrounding nonenhancing expansile infiltrative changes. Ring-enhancing lesions can be seen in patients with anaplastic ependymoma, but is not commonly reported in grade II ependymomas. In summary, this report highlights a unique case of a posterior fossa ependymoma in a pediatric patient arising in an atypical brainstem location as well as having unique imaging features.
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Ependimoma , Terapia com Prótons , Biópsia , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/cirurgia , Criança , Ependimoma/diagnóstico por imagem , Ependimoma/cirurgia , Quarto Ventrículo/diagnóstico por imagem , Quarto Ventrículo/cirurgia , Humanos , MasculinoRESUMO
Our purpose was to analyze the pattern of failure in glioblastoma (GBM) patients at first recurrence after radiotherapy and temozolomide and its relationship with different factors. From 77 consecutive GBM patients treated at our institution with fluorescence guided surgery and standard radiochemotherapy, 58 first recurrences were identified and included in a retrospective review. Clinical data including age, Karnofsky performance score, preoperative tumor volume and location, extend of resection, MGMT promoter methylation status, time to progression (PFS), overall survival (OS) and adjuvant therapies were reviewed for every patient. Recurrent tumor location respect the original lesion was the end point of the study. The recurrence pattern was local only in 65.5% of patients and non-local in 34.5%. The univariate and multivariate analysis showed that greater preoperative tumor volume in T1 gadolinium enhanced sequences, was the only variable with statistical signification (p < 0.001) for increased rate of non-local recurrences, although patients with MGMT methylation and complete resection of enhancing tumor presented non-local recurrences more frequently. PFS was longer in patients with non-local recurrences (13.8 vs. 6.4 months; p = 0.019, log-rank). However, OS was not significantly different in both groups (24.0 non-local vs. 19.3 local; p = 0.9). Rate of non-local recurrences in our series of patients treated with fluorescence guided surgery and standard radiochemotherapy was higher than previously published in GBM, especially in patients with longer PFS. Greater preoperative enhancing tumor volume was associated with increased rate of non-local recurrences.
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Neoplasias Encefálicas/terapia , Glioblastoma/terapia , Recidiva Local de Neoplasia/etiologia , Antineoplásicos Alquilantes/efeitos adversos , Neoplasias Encefálicas/genética , Metilases de Modificação do DNA/genética , Enzimas Reparadoras do DNA/genética , Dacarbazina/efeitos adversos , Dacarbazina/análogos & derivados , Progressão da Doença , Feminino , Glioblastoma/genética , Humanos , Avaliação de Estado de Karnofsky , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Recidiva Local de Neoplasia/genética , Regiões Promotoras Genéticas/genética , Radioterapia/efeitos adversos , Temozolomida , Proteínas Supressoras de Tumor/genéticaRESUMO
OBJECTIVE: Patients who experience postoperative pediatric cerebellar mutism syndrome (CMS) during treatment for medulloblastoma have long-term deficits in neurocognitive functioning; however, the consequences on functional or adaptive outcomes are unknown. The purpose of the present study was to compare adaptive, behavioral, and emotional functioning between survivors with and those without a history of CMS. METHODS: The authors examined outcomes in 45 survivors (15 with CMS and 30 without CMS). Comprehensive neuropsychological evaluations, which included parent-report measures of adaptive, behavioral, and emotional functioning, were completed at a median of 2.90 years following craniospinal irradiation. RESULTS: Adaptive functioning was significantly worse in the CMS group for practical and general adaptive skills compared with the group without CMS. Rates of impairment in practical, conceptual, and general adaptive skills in the CMS group exceeded expected rates in the general population. Despite having lower overall intellectual functioning, working memory, and processing speed, IQ and related cognitive processes were uncorrelated with adaptive outcomes in the CMS group. No significant group differences or increased rates of impairment were observed for behavioral and emotional outcomes. CONCLUSIONS: Survivors with CMS, compared with those without CMS, are rated as having significant deficits in overall or general adaptive functioning, with specific weakness in practical skills several years posttreatment. Findings from this study demonstrate the high risk for ongoing functional deficits despite acute recovery from symptoms of CMS, highlighting the need for intervention to mitigate such risk.
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Adaptação Psicológica , Neoplasias Cerebelares , Meduloblastoma , Mutismo , Humanos , Meduloblastoma/cirurgia , Meduloblastoma/radioterapia , Meduloblastoma/psicologia , Meduloblastoma/complicações , Masculino , Feminino , Criança , Mutismo/etiologia , Mutismo/psicologia , Neoplasias Cerebelares/cirurgia , Neoplasias Cerebelares/psicologia , Neoplasias Cerebelares/radioterapia , Neoplasias Cerebelares/complicações , Adolescente , Emoções , Testes Neuropsicológicos , Complicações Pós-Operatórias/psicologia , Complicações Pós-Operatórias/etiologia , Pré-EscolarRESUMO
PURPOSE: T cells modified with chimeric antigen receptors (CARTs) have demonstrated efficacy for hematologic malignancies; however, benefit for patients with CNS tumors has been limited. To enhance T cell activity against GD2+ CNS malignancies, we modified GD2-directed CART cells (GD2.CARTs) with a constitutively active interleukin (IL)-7 receptor (C7R-GD2.CARTs). METHODS: Patients age 1-21 years with H3K27-altered diffuse midline glioma (DMG) or other recurrent GD2-expressing CNS tumors were eligible for this phase I trial (ClinicalTrials.gov identifier: NCT04099797). All subjects received standard-of-care adjuvant radiation therapy or chemotherapy before study enrollment. The first treatment cohort received GD2.CARTs alone (1 × 107 cells/m2), and subsequent cohorts received C7R-GD2.CARTs at two dose levels (1 × 107 cells/m2; 3 × 107 cells/m2). Standard lymphodepletion with cyclophosphamide and fludarabine was included at all dose levels. RESULTS: Eleven patients (age 4-18 years) received therapy without dose-limiting toxicity. The GD2.CART cohort did not experience toxicity, but had disease progression after brief improvement of residual neurologic deficits (≤3 weeks). The C7R-GD2.CART cohort developed grade 1 tumor inflammation-associated neurotoxicity in seven of eight (88%) cases, controllable with anakinra. Cytokine release syndrome was observed in six of eight (75%, grade 1 in all but one patient) and associated with increased circulating IL-6 and IP-10 (P < .05). Patients receiving C7R-GD2.CARTs experienced temporary improvement from baseline neurologic deficits (range, 2 to >12 months), and seven of eight (88%) remained eligible for additional treatment cycles (range 2-4 cycles). Partial responses by iRANO criteria were observed in two of seven (29%) patients with DMG treated by C7R-GD2.CARTs. CONCLUSION: Intravenous GD2.CARTs with and without C7R were well tolerated. Patients treated with C7R-GD2.CARTs exhibited transient improvement of neurologic deficits and increased circulating cytokines/chemokines. Treatment with C7R-GD2.CARTs represents a novel approach warranting further investigation for children with these incurable CNS cancers.
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Neoplasias do Sistema Nervoso Central , Gangliosídeos , Receptores de Antígenos Quiméricos , Humanos , Criança , Adolescente , Pré-Escolar , Masculino , Feminino , Receptores de Antígenos Quiméricos/imunologia , Receptores de Antígenos Quiméricos/uso terapêutico , Lactente , Adulto Jovem , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/imunologia , Neoplasias do Sistema Nervoso Central/terapia , Gangliosídeos/imunologia , Imunoterapia Adotiva/efeitos adversos , Imunoterapia Adotiva/métodos , Gradação de Tumores , Glioma/tratamento farmacológico , Glioma/patologia , Glioma/terapia , Glioma/imunologia , Subunidade alfa de Receptor de Interleucina-7RESUMO
INTRODUCTION: Muir-Torre syndrome is a genetic disease characterised by the association of sebaceous neoplasms with visceral neoplasms, mainly colorectal cancer and secondly urogenital tumours. Metastases from prostate tumours without systemic disease are rare in the brain and exceptional in the brainstem. CASE REPORT: We present a 48-year old male, with a single brainstem metastasis from a prostate adenocarcinoma, who had previously been diagnosed with Muir-Torre syndrome. Diagnostic stereotactic biopsy was performed. CONCLUSION: Single metastasis from a prostate adenocarcinoma in the brainstem without systemic disease is exceptional. Due to the different diagnostic possibilities, biopsy should be performed in order to obtain a diagnosis, especially in the context of Muir-Torre syndrome.
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Adenocarcinoma/secundário , Biópsia , Neoplasias do Tronco Encefálico/secundário , Síndrome de Muir-Torre , Neoplasias da Próstata/patologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/genética , Adenocarcinoma/terapia , Adenoma/genética , Anilidas/administração & dosagem , Anilidas/uso terapêutico , Antineoplásicos Hormonais/administração & dosagem , Antineoplásicos Hormonais/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias do Tronco Encefálico/diagnóstico , Neoplasias do Tronco Encefálico/genética , Terapia Combinada , Docetaxel , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Muir-Torre/genética , Proteína 2 Homóloga a MutS/deficiência , Proteína 2 Homóloga a MutS/genética , Mutação , Neoplasias Primárias Múltiplas/genética , Neuronavegação/métodos , Nitrilas/administração & dosagem , Nitrilas/uso terapêutico , Prostatectomia , Neoplasias da Próstata/genética , Neoplasias da Próstata/terapia , Radioterapia Adjuvante , Neoplasias Retais/genética , Neoplasias do Colo Sigmoide/genética , Taxoides/administração & dosagem , Compostos de Tosil/administração & dosagem , Compostos de Tosil/uso terapêuticoRESUMO
Background: Gangliogliomas (GGs) are rare tumors of the central nervous system composed of neoplastic neural and glial cells and are typically low-grade. Intramedullary spinal anaplastic GGs (AGG) are rare, poorly understood, and often aggressive tumors that can result in widespread progression along the craniospinal axis. Due to the rarity of these tumors, data are lacking to guide clinical and pathologic diagnosis and standard of care treatment. Here, we present a case of pediatric spinal AGG to provide information on our institutional approach to work-up and to highlight unique molecular pathology. Case Description: A 13-year-old female presented with signs of spinal cord compression including right sided hyperreflexia, weakness, and enuresis. Magnetic resonance imaging (MRI) revealed a C3-C5 cystic and solid mass which was treated surgically with osteoplastic laminoplasty and tumor resection. Histopathologic diagnosis was consistent with AGG, and molecular testing identified mutations in H3F3A (K27M), TP53, and NF1. She received adjuvant radiation therapy and her neurological symptoms improved. However, at 6-month follow-up, she developed new symptoms. MRI revealed metastatic recurrence of tumor with leptomeningeal and intracranial spread. Conclusion: Primary spinal AGGs are rare tumors, but a growing body of literature shows some trends that may improve diagnosis and management. These tumors generally present in adolescence and early adulthood with motor/sensory impairment and other spinal cord symptoms. They are most commonly treated by surgical resection but frequently recur due to their aggressive nature. Further reports of these primary spinal AGGs along with characterization of their molecular profile will be important in developing more effective treatments.
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OBJECTIVE: The role of surgery in recurrent ependymomas and its contribution to the outcome are not well defined. While gross-total resection (GTR) has shown benefit in newly diagnosed patients with improvement in progression-free survival (PFS), its impact after recurrence is not known. Its role in distant relapses or multiple local recurrences is similarly less well understood. The objective of this study was to investigate whether GTR could prolong survival after recurrence. METHODS: In this paper, the authors identified patients with ependymomas who underwent surgery at Texas Children's Hospital for recurrent ependymomas between December 2000 and December 2021. Surgical treatment was stratified as GTR, subtotal resection (STR), or a biopsy. Kaplan-Meier analysis was performed for PFS and overall survival (OS), and the log-rank test was used to assess statistical significance. The Cox regression model was used for multivariable analysis. RESULTS: Forty children were identified with a first ependymoma recurrence and follow-up data were collected. The median age was 5.46 years (95% CI 4.52-6.39 years) with a mean follow-up of 3.92 years (95% CI 2.42-5.42 years). In 26 patients (65%), the original tumor was located in the infratentorial space. Twenty-nine patients (72.5%) presented with local recurrence. Within this group, the 5-year PFS rates for the GTR and STR groups were 40.1% and 26.8%, respectively. The 5- and 10-year OS rates were 58.3% and 50% in the GTR group and 51% and 16.7% in the STR group, respectively. Fifteen patients presented with a second recurrence. The 5-year PFS and OS rates in patients who had GTR after a second recurrence were 33% and 50%, respectively. CONCLUSIONS: GTR of local recurrent ependymomas can result in long-term survival in first and second recurrences. Further and larger studies are necessary to elucidate the role of surgery in distal recurrences.
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Ependimoma , Recidiva Local de Neoplasia , Criança , Humanos , Pré-Escolar , Recidiva Local de Neoplasia/cirurgia , Procedimentos Neurocirúrgicos , Estudos Retrospectivos , Resultado do Tratamento , Ependimoma/cirurgia , Ependimoma/diagnóstico , RecidivaRESUMO
OBJECTIVE: The optimal management of pediatric craniopharyngioma patients remains controversial, shifting from radical resection (gross-total resection [GTR]) to a more conservative approach with partial resection/biopsy followed by radiotherapy (PR+RT). To the authors' knowledge, no previous studies have compared neurocognitive and quality-of-life (QOL) outcomes between the two main treatments. In this study, the authors compared changes in intellectual, adaptive, and QOL scores in children treated for craniopharyngioma with GTR and those treated with PR+RT. METHODS: Patients underwent annual neurocognitive and QOL evaluations for up to 10 years posttreatment, including the Full-Scale IQ, Verbal Comprehension Index (VCI), Perceptual Reasoning Index (PRI), Working Memory Index (WMI), and Processing Speed Index (PSI). Child- and parent-reported QOL scores and adaptive behavior in different domains were assessed. General linear mixed models were used to examine change in scores over time by treatment group with adjustment for significant covariates. RESULTS: Scores from 43 patients treated between 2009 and 2019 (21 GTR, 22 PR+RT) were examined. Within the PR+RT group, 9 patients had intensity-modulated RT and 13 had proton beam therapy. The treatment groups were similar in sex (44% male) and age (median 7.3 years). There were no significant differences in the trajectory of intellectual functioning or QOL scale scores between the two groups. However, patients who underwent GTR exhibited significant improvement over time in overall adaptive behavior (p = 0.04) and conceptual skills (p = 0.01), which was not observed in patients treated with PR+RT. CONCLUSIONS: Long-term pediatric craniopharyngioma survivors treated with GTR and PR+RT have similar intellectual function and QOL. Larger studies are needed to explore small but clinically significant differences between the two groups.
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Craniofaringioma , Neoplasias Hipofisárias , Terapia com Prótons , Humanos , Masculino , Criança , Feminino , Craniofaringioma/cirurgia , Qualidade de Vida , Neoplasias Hipofisárias/cirurgia , Radioterapia Adjuvante , Resultado do Tratamento , Estudos RetrospectivosRESUMO
OBJECTIVE: In utero repair of fetal posterior cephaloceles (meningocele and encephalocele) is being performed based on the premise that fetal surgery prevents progressive herniation of neural tissue and brain damage during pregnancy. However, the extent to which progressive herniation occurs during pregnancy, specifically from prenatal diagnosis to after delivery, is not well known. The objective of this study was to describe the natural history of patients with fetal cephaloceles focusing on the incidence of progressive herniation. METHODS: The authors conducted a retrospective cohort study of all patients referred to their center for posterior fetal cephalocele between 2006 and 2021. All patients underwent prenatal and postnatal MRI. Progressive herniation (primary outcome) was defined as an increase in the absolute volume of neural tissue within the cephalocele of > 5% or new herniation of a critical structure into the cephalocele. Total brain and cephalocele volumes were calculated to determine herniation progression from prenatal to postnatal MRI. Information on the presence of hydrocephalus, epilepsy, and developmental delay (secondary outcomes) was collected at 1 year of age. RESULTS: Twenty patients met all study criteria. Ten patients (50%; 95% CI 0.27-0.73) demonstrated progressive herniation from prenatal to postnatal MRI. Three patients with progressive herniation were diagnosed with a meningocele prenatally and had an encephalocele postnatally. Two patients without progression had meningocele identified prenatally that regressed and became atretic by birth. Both prenatal hindbrain herniation (p = 0.03) and prenatal microcephaly (p = 0.05) were predictive of progressive herniation. The rates of hydrocephalus (44%), epilepsy (44%), and developmental delay (63%) were not associated with the occurrence of progressive herniation in this study. CONCLUSIONS: In this study, progressive herniation was not a rare event (50%). Fetal hindbrain herniation and fetal microcephaly were associated with the occurrence of progressive herniation. These results support further investigations into why progressive herniation occurs in utero and if progressive cerebral herniation in utero plays a significant role in determining clinical outcome.
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Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, occurs in a subset of children after posterior fossa tumor resection, most commonly medulloblastoma. Patients with this syndrome exhibit often transient, although protracted, symptoms of language impairment, emotional lability, cerebellar, and brainstem dysfunction. However, many patients experience persistent neurological deficits and lasting neurocognitive impairment. Historically, research and clinical care were hindered by inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty surrounding risk factors and etiology. Proposed diagnostic criteria include two major symptoms, language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement as well as other experts in this field. Risk factors most commonly associated with development of CMS include midline tumor location, diagnosis of medulloblastoma and specific tumor subtype, younger age at diagnosis, and preoperative language impairment. A proposed etiology of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Treatment for CMS remains supportive. Herein, we present a comprehensive overview of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. In addition, we identify essential multidisciplinary research priorities to advance diagnostics, prevention, and intervention efforts for patients with, or at risk for, development of CMS.
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Doenças Cerebelares , Neoplasias Cerebelares , Transtornos do Desenvolvimento da Linguagem , Meduloblastoma , Mutismo , Doenças Cerebelares/complicações , Doenças Cerebelares/diagnóstico , Neoplasias Cerebelares/complicações , Criança , Humanos , Meduloblastoma/complicações , Meduloblastoma/diagnóstico , Meduloblastoma/terapia , Mutismo/diagnóstico , Mutismo/etiologia , Mutismo/terapia , Complicações Pós-Operatórias , Pesquisa , SíndromeRESUMO
Pineal region tumors represent a formidable challenge to the neurosurgeon. Choosing the right approach is key to optimizing the extent of resection and minimizing surgical morbidity. In this video, the authors show an interhemispheric transcallosal approach to a pineal region tumor in a 15-year-old boy. The advantage of this corridor over posterior approaches is that it provides a nice view of the tumor plane with the venous complex, especially while dissecting tumor from the anterior aspect of the internal cerebral veins on their vertical path. Thus, this approach represents a safe and effective alternative for selected pineal tumors. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2120.
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Texas Children's Hospital opened its doors in 1954, and since that time the institution has remained dedicated to a three-part mission: patient care, education, and research. Dr. William R. Cheek developed an early interest in pediatric neurosurgery, which led to his efforts in building and developing a service at Texas Children's Hospital at a time when the field was just emerging. His work with other early pioneers in the field led to the establishment of organized societies, educational texts, and governing bodies that have led to significant advances in the field over the past 50 years.
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BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare but potentially morbid disease in the pediatric population, and the optimal treatment is not fully understood. Endovascular intervention for this condition has been rarely reported. METHODS: The Stroke Thrombectomy and Aneurysm Registry was queried for patients aged less than or equal to 18 years undergoing endovascular treatment for CVST in the past 10 years. Clinical charts and radiographic data were retrospectively reviewed. Modified Rankin Score (mRS) at 90 days postprocedure was determined as the primary outcome. RESULTS: A total of seven patients across five pediatric centers ranging from 7 to 16 years of age were identified with a mean follow-up of 28 months. All had underlying conditions predisposing to CVST. Endovascular intervention was undertaken due to neurological deterioration despite systemic anticoagulation; venous infarct was evident preoperatively in six of seven patients. Mechanical venous thrombectomy was attempted in all individuals, and intrasinus thrombolytic therapy was also performed in three cases. Six patients had favorable outcome with mRS 0 or 1 at 90 days postprocedure; one remained neurologically devastated. CONCLUSIONS: Endovascular treatment by an experienced interventionalist may be safe and effective in severe cases of CVST in children failing frontline therapy. Children with radiographic or clinical progression despite anticoagulation may be considered for endovascular intervention in a timely manner.
Assuntos
Procedimentos Endovasculares , Fibrinolíticos/administração & dosagem , Trombólise Mecânica , Avaliação de Resultados em Cuidados de Saúde , Sistema de Registros , Trombose dos Seios Intracranianos/terapia , Adolescente , Criança , Feminino , Seguimentos , Humanos , Masculino , Recidiva , Estudos RetrospectivosRESUMO
OBJECTIVE: The authors compared survival and multiple comorbidities in children diagnosed with craniopharyngioma who underwent gross-total resection (GTR) versus subtotal resection (STR) with radiation therapy (RT), either intensity-modulated radiation therapy (IMRT) or proton beam therapy (PBT). The authors hypothesized that there are differences between multimodal treatment methods with respect to morbidity and progression-free survival (PFS). METHODS: The medical records of children diagnosed with craniopharyngioma and treated surgically between February 1997 and December 2018 at Texas Children's Hospital were reviewed. Surgical treatment was stratified as GTR or STR + RT. RT was further stratified as PBT or IMRT; PBT was stratified as STR + PBT versus cyst decompression (CD) + PBT. The authors used Kaplan-Meier analysis to compare PFS and overall survival, and chi-square analysis to compare rates for hypopituitarism, vision loss, and hypothalamic obesity (HyOb). RESULTS: Sixty-three children were included in the analysis; 49% were female. The mean age was 8.16 years (95% CI 7.08-9.27). Twelve of 14 children in the IMRT cohort underwent CD. The 5-year PFS rates were as follows: 73% for GTR (n = 31), 54% for IMRT (n = 14), 100% for STR + PBT (n = 7), and 77% for CD + PBT (n = 11; p = 0.202). The overall survival rates were similar in all groups. Rates of hypopituitarism (96% GTR vs 75% IMRT vs 100% STR + PBT, 50% CD + PBT; p = 0.023) and diabetes insipidus (DI) (90% GTR vs 61% IMRT vs 85% STR + PBT, 20% CD + PBT; p = 0.004) were significantly higher in the GTR group. There was no significant difference in the HyOb or vision loss at the end of study follow-up among the different groups. Within the PBT group, 2 patients presented a progressive vasculopathy with subsequent strokes. One patient experienced a PBT-induced tumor. CONCLUSIONS: GTR and CD + PBT presented similar rates of 5-year PFS. Hypopituitarism and DI rates were higher with GTR, but the rate of HyOb was similar among different treatment modalities. PBT may reduce the burden of hypopituitarism and DI, although radiation carries a risk of potential serious complications, including progressive vasculopathy and secondary malignancy. Further prospective study comparing neurocognitive outcomes is necessary.