O glioma das vias ópticas (GVO) é um tumor benigno (não-canceroso) que cresce no nervo óptico e nas suas "extensões" (partes como o quiasma, os tratos e as radiações, que formam todo o caminho da visão até o cérebro). Ele é caracterizado por causar problemas ou a perda da visão, e também pode vir junto com outros sintomas, como um crescimento mais lento e alterações no sono. Esses últimos sintomas estão ligados a uma parte específica do cérebro. O GVO é frequentemente associado à neurofibromatose tipo 1 (NF1), uma condição genética.
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O glioma das vias ópticas (GVO) é um tumor benigno (não-canceroso) que cresce no nervo óptico e nas suas "extensões" (partes como o quiasma, os tratos e as radiações, que formam todo o caminho da visão até o cérebro). Ele é caracterizado por causar problemas ou a perda da visão, e também pode vir junto com outros sintomas, como um crescimento mais lento e alterações no sono. Esses últimos sintomas estão ligados a uma parte específica do cérebro. O GVO é frequentemente associado à neurofibromatose tipo 1 (NF1), uma condição genética.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 5 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 23 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Glioma das vias ópticas
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Publicações mais relevantes
Differences in Optic Pathway Glioma Prevalence among Children with Neurofibromatosis Type 1.
This multi-institutional, natural language processing-based analysis of 273 children with neurofibromatosis 1-associated optic pathway gliomas examines the impact of ancestral background on clinical presentation and progression. We report that White children had higher OPG prevalence than Black or Asian children, but clinical features and outcomes did not differ across groups.
Central Precocious Puberty and Optic Pathway Glioma in Children with Neurofibromatosis 1: Associations with Tumor Location, Vision, and Treatment.
Neurofibromatosis 1-associated optic pathway gliomas (OPGs) may co-occur with central precocious puberty (CPP). Using large language model-based data analysis from 2 NF centers, we compared OPGs in children with NF1, with and without CPP. CPP was associated with hypothalamic involvement, poorer vision, and a higher likelihood of OPG treatment.
Frontotemporal-orbitozygomatic approach for pediatric cranio-orbital tumors: a single-center experience.
To evaluate the surgical anatomy, technical nuances, and early postoperative outcomes of the frontotemporal-orbitozygomatic (FTOZ) approach in a consecutive series of pediatric cranio-orbital communicating tumors. To evaluate the surgical anatomy, technical nuances, and early postoperative outcomes of the frontotemporal-orbitozygomatic (FTOZ) approach in a consecutive series of pediatric cranio-orbital communicating tumors. Unilateral Dodge Class I Optic Pathway Glioma without Neurofibromatosis Type 1 (OPG-uDCI) was selected as an index pathology to illustrate the specific application of this approach in a representative subgroup. Thirteen children (five female, eight male) with imaging and histopathology confirmed cranio-orbital communicating tumours were managed at a single institution from April 2020 to August 2024. At operation, the median age was 24 months (interquartile range 4-156 months). Postoperative surveillance extended for a median of 21 months (interquartile range 9-62 months). Notably, 10 patients had a follow-up period of at least 12 months. Histopathological diagnoses included: OPG, rhabdomyosarcoma, mature teratoma, optic nerve sheath meningioma, langerhans cell histiocytosis, and ewing-like sarcoma. Surgical complications included one case of severe postoperative chemosis with corneal ulceration requiring enucleation and one wound infection. There were no operative mortalities in this series. During follow-up, no patients with OPG demonstrated contralateral visual field changes postoperatively. Among the seven participants in whom macroscopic total excision was accomplished, neoplastic relapse subsequently developed in three. The FTOZ approach is a technically feasible corridor for resection of pediatric cranio-orbital tumors in selected cases. However, given the small sample size, short follow-up, and occurrence of major complications, these findings should be considered preliminary. Definitive conclusions regarding safety and efficacy await larger comparative studies.
Using Optical Coherence Tomography in Pediatric Neuro-ophthalmology.
Optical coherence tomography (OCT) has transformed adult neuro-ophthalmology, but pediatric adoption has lagged because of perceived technical, financial, and practical barriers. This chapter provides a pragmatic framework for integrating OCT into pediatric and pediatric neuro-ophthalmology clinics, emphasizing how to obtain reliable images in awake children and how to interpret them. We first review technical aspects of tabletop and handheld OCT in children, including positioning, fixation strategies, and when to prioritize single-line macular or optic nerve head B-scans over full segmented protocols. We then outline key clinical modalities: single-line foveal scans for "in vivo histology" and ganglion cell layer/inner nuclear layer (GCL/INL) ratio screening; single-line optic nerve head (ONH) scans to assess Bruch membrane contour and opening size; ONH cube maps for papilledema versus pseudopapilledema; macular cube maps with segmentation to detect retinotopic patterns of GCL loss; and strategies to correct RNFL interpretation for axial length. Using a structured table-based approach, we show how combined RNFL and GCL measurements narrow the differential diagnosis and guide urgency and systemic workup in children with decreased vision and normal, swollen, or atrophic optic nerves. Disease-focused sections illustrate OCT applications in papilledema, pseudopapilledema, optic neuritis, neuroretinitis, optic pathway glioma, optic atrophy (including INL microcysts), infantile nystagmus, and congenital optic nerve anomalies. Throughout, we highlight pitfalls such as pseudonormalization of the RNFL, segmentation errors in high-grade edema or anomalous discs, and misinterpretation in highly ametropic eyes. The chapter aims to make OCT a routine, child-friendly extension of the neuro-ophthalmic exam.
Diencephalic Syndrome: Clinical Features, Pathophysiology, and Long-Term Outcomes.
Background/Objectives: Diencephalic syndrome (DS) is an uncommon pediatric disorder presenting with severe failure to thrive despite adequate caloric intake and preserved linear growth. First characterized by Russell, this condition predominantly affects infants under 12 months and remains diagnostically challenging. Methods: We performed a comprehensive literature review examining clinical presentation, underlying pathophysiology, associated pathology, diagnostic approaches, and long-term outcomes of DS. Results: DS typically manifests in the first year of life with profound cachexia, normal or increased appetite, preserved height velocity, and characteristic features including hyperactivity, euphorism, and visual pathway involvement. Low-grade gliomas of the hypothalamic-chiasmatic region, particularly pilocytic astrocytomas, represent the predominant underlying pathology. The pathophysiological mechanisms remain incompletely understood but likely involve complex dysregulation of hypothalamic energy homeostasis. While overall survival exceeds 90% at five years, most patients experience significant long-term morbidity including visual impairment, multiple endocrine deficiencies, and hypothalamic obesity. Diagnostic delays averaging 11 months contribute to irreversible complications. Conclusions: Early recognition of DS is essential to prevent permanent visual loss and optimize outcomes. Multidisciplinary management incorporating chemotherapy as first-line treatment for underlying gliomas has improved survival while reducing radiation-associated toxicities. However, survivors face substantial lifelong sequelae requiring comprehensive monitoring and intervention. Future research should focus on elucidating precise pathophysiological mechanisms, developing targeted molecular therapies, and improving management of hypothalamic obesity and other late effects.
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Journal of neuro-oncologyPredictive Modeling for Clinical Features Associated With Neurofibromatosis Type 1.
Neurology. Clinical practice[Prognostic factors of neurofibromatosis type 1-associated optic pathway gliomas in children].
Journal francais d'ophtalmologieThe diagnostic accuracy and prognostic value of OCT for the evaluation of the visual function in children with a brain tumour: A systematic review.
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Nature communicationsNeuroimaging Features of Optic Nerve Hemangioblastoma Identified by Conventional and Advanced Magnetic Resonance Techniques: A Case Report and Literature Review.
Frontiers in oncologyOptic pathway glioma and the sex association in neurofibromatosis type 1: a single-center study.
Orphanet journal of rare diseasesRegression and pseudoprogression of pediatric optic pathway glioma in patients treated with proton beam therapy.
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Developmental cellImpact of systemic anticancer therapy in pediatric optic pathway glioma on visual function: A systematic review.
PloS oneNovel Variants and Clinical Characteristics of 16 Patients from Southeast Asia with Genetic Variants in Neurofibromin-1.
Journal of pediatric geneticsA Unique Case of Sporadic Optic Pathway Glioma in an Infant With Acute Nystagmus.
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Molecular genetics & genomic medicineCase Report: Chemotherapy Indication in a Case of Neurofibromatosis Type 1 Presenting Optic Pathway Glioma: A One-Year Clinical Case Study Using Differential Tractography Approach.
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JPMA. The Journal of the Pakistan Medical AssociationClinical spectrum of neurofibromatosis type 1 among children in a tertiary care center.
Neurosciences (Riyadh, Saudi Arabia)C6 Cell Injection into the Optic Nerve of Long-Evans Rats: A Short-Term Model of Optic Pathway Gliomas.
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NeuroImage. ClinicalThe Visual Acuity Outcome and Relevant Factors Affecting Visual Improvement in Pediatric Sporadic Chiasmatic-Hypothalamic Glioma Patients Who Received Surgery.
Frontiers in neurologyPredicting pediatric optic pathway glioma progression using advanced magnetic resonance image analysis and machine learning.
Neuro-oncology advancesMalignant optic nerve glioma in a young woman with 7 year follow up without recurrence.
American journal of ophthalmology case reportsLow-Grade Gliomas in Patients with Noonan Syndrome: Case-Based Review of the Literature.
Diagnostics (Basel, Switzerland)Visual function tests including the role of optical coherence tomography in neurofibromatosis 1.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryEffect of age and neurofibromatosis type 1 status on white matter integrity in the optic radiations.
Neuro-oncology advancesNF1-like optic pathway gliomas in children: clinical and molecular characterization of this specific presentation.
Neuro-oncology advancesNF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat.
Neuro-oncologyLoss of efficacy of subsequent nonsurgical therapy after primary treatment failure in pediatric low-grade glioma patients-Report from the German SIOP-LGG 2004 cohort.
International journal of cancerNeurofibromatosis 1-associated optic pathway gliomas.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryIntraoperative visual evoked potential monitoring for optic pathway glioma in an infant: A case description.
Clinical neurophysiology : official journal of the International Federation of Clinical NeurophysiologyNeurofibromatosis type 1-related hydrocephalus: causes and treatment considerations.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryRetrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1.
CancersHomonymous Retinal Ganglion Cell Layer Atrophy With Asymptomatic Optic Tract Glioma in Neurofibromatosis Type I.
Frontiers in neurologyNonoptic pathway tumors in children with neurofibromatosis type 1.
NeurologyPretreatment Endocrine Disorders Due to Optic Pathway Gliomas in Pediatric Neurofibromatosis Type 1: Multicenter Study.
The Journal of clinical endocrinology and metabolismNeurofibromatosis Type 1-Associated Optic Pathway Glioma in Children: A Follow-Up of 10 Years or More.
American journal of ophthalmologyVisual field fluctuations correlating with the menstrual cycle in a patient with optic pathway glioma.
BMJ case reportsOptic pathway glioma with intraocular extension in a child with neurofibromatosis type-1.
Journal francais d'ophtalmologieExpanded transnasal approaches to the skull base in the Middle East: Where do we stand?
Annals of Saudi medicine3 Tesla MRI brain scanning under general anaesthesia in a paediatric 3 Tesla-compatible cochlear implant recipient, first reported case: Clinical considerations and implications for future practice.
International journal of pediatric otorhinolaryngologyNeuropsychological outcomes of children with Optic Pathway Glioma.
Scientific reportsRole of visual evoked potentials and optical coherence tomography in the screening for optic pathway gliomas in patients with neurofibromatosis type I.
European journal of ophthalmology[Effect of bevacizumab in treatment of children with optic pathway glioma].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsRadiation-Induced Moyamoya Syndrome in Children with Brain Tumors: Case Series and Literature Review.
World neurosurgeryRisk of Optic Pathway Glioma in Neurofibromatosis Type 1: No Evidence of Genotype-Phenotype Correlations in A Large Independent Cohort.
CancersOptic Pathway Glioma in Type 1 Neurofibromatosis: Review of Its Pathogenesis, Diagnostic Assessment, and Treatment Recommendations.
CancersBrain Tumors in NF1 Children: Influence on Neurocognitive and Behavioral Outcome.
CancersLymphocyte Subpopulations in Patients With Neurofibromatosis Type 1-associated Optic Pathway Gliomas and Plexiform Neurofibromas.
Anticancer researchBevacizumab for optic pathway glioma with worsening visual field in absence of imaging progression: 2 case reports and literature review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAssessment of the visual pathways in patients with neurofibromatosis-1 by 3S-space technique with 3-Tesla MRI.
Turkish journal of medical sciencesDevelopment of a multidisciplinary clinic of neurofibromatosis type 1 and other neurocutaneous disorders in Greece. A 3-year experience.
Postgraduate medicineNeurological malignancies in neurofibromatosis type 1.
Current opinion in oncologyLong-term visual acuity outcomes after radiation therapy for sporadic optic pathway glioma.
Journal of neuro-oncologyRare Case of Sporadic Malignant Optic Pathway Glioma in 71-Year-Old Woman.
World neurosurgery68Ga-NOTA-Aca-BBN(7-14) PET imaging of GRPR in children with optic pathway glioma.
European journal of nuclear medicine and molecular imagingCentral and peripheral steady-state visual evoked potentials in children with optic pathway gliomas.
Documenta ophthalmologica. Advances in ophthalmologyAcromegaly caused by a somatotroph adenoma in patient with neurofibromatosis type 1.
Endocrine journalInfantile Optic Pathway Glioblastoma.
World neurosurgeryNeurofibromatosis Type 1-Related Hydrocephalus: Treatment Options and Considerations.
World neurosurgeryRetinal Vascular and Neural Remodeling Secondary to Optic Nerve Axonal Degeneration: A Study Using OCT Angiography.
Ophthalmology. RetinaAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Differences in Optic Pathway Glioma Prevalence among Children with Neurofibromatosis Type 1.
- Central Precocious Puberty and Optic Pathway Glioma in Children with Neurofibromatosis 1: Associations with Tumor Location, Vision, and Treatment.
- Frontotemporal-orbitozygomatic approach for pediatric cranio-orbital tumors: a single-center experience.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2026· PMID 41870661mais citado
- Using Optical Coherence Tomography in Pediatric Neuro-ophthalmology.
- Diencephalic Syndrome: Clinical Features, Pathophysiology, and Long-Term Outcomes.
- Progressive cervicothoracic meningocele with neurovascular compression and spinal deformity in neurofibromatosis type 1: a decade-long radiological and clinical evolution with operative repair. Illustrative case.
- Coexisting Optic Pathway and Posterior Fossa Gliomas in an Adult With Previously Undiagnosed Neurofibromatosis Type 1.
- Dynamic contrast-enhanced magnetic resonance imaging in paediatric brain tumours systematically reviewed.
- Extended endoscopic endonasal transplanum-transtuberculum surgery for optic pathway-hypothalamic glioma presenting with tonsillar herniation and syringomyelia: illustrative case.
- Pediatric orbital masses from a tertiary pediatric oncology referral center: clinical characteristics and survival outcomes from a 20-year single-center experience.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:2086(Orphanet)
- MONDO:0016167(MONDO)
- GARD:4107(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q7936613(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
