scholarly journals Tumor load rather than contrast enhancement is associated with the visual function of children and adolescents with optic pathway glioma – a retrospective Magnetic Resonance Imaging study

2022 ◽  
Author(s):  
Anna Kilian ◽  
Annette Aigner ◽  
Michèle Simon ◽  
Daniel J. Salchow ◽  
Cornelia Potratz ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Contrast Enhancement ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Rank Correlation ◽  
Imaging Study ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Tumor Load ◽  
Optic Pathway Gliomas

Abstract Introduction Optic pathway gliomas are often asymptomatic tumors occurring in children with neurofibromatosis type 1 (NF1 + OPG) or sporadically (spOPG). Treatment is usually prompted by visual loss and/or tumor progression on MRI. The aim of this study was to investigate the relationship between visual acuity (VA), tumor growth, and contrast enhancement to provide more distinct indications for the administration of gadolinium-based contrast agents. Methods Tumor load was retrospectively measured and enhancement semi-quantitatively scored on 298 MRIs of 35 patients (63% NF1 + OPG). Spearman rank correlation between tumor load and enhancement was calculated and a linear mixed model used to examine the influence of tumor load and enhancement on corresponding VA tests (LogMAR). Results The optic nerve width in NF1 + OPGs was strongly associated with VA (regression coefficient 0.75; confidence interval 0.61—0.88), but weakly with enhancement (0.06; −0.04—0.15). In spOPGs, tumor volume and optic nerve width were more relevant (0.31; −0.19—0.81 and 0.39; 0.05—0.73) than enhancement (0.09; −0.09—0.27). Conclusions Tumor load measures may be more relevant for the surveillance of optic pathway gliomas than enhancement, given that VA is the relevant outcome parameter. Regular contrast administration should therefore be questioned in these patients.

scholarly journals Neuroophthalmological management of optic pathway gliomas

2007 ◽  
Vol 23 (5) ◽  
pp. E1 ◽  
Author(s):  
Andrew G. Lee
Keyword(s):  
Growth Rate ◽  
Optic Nerve ◽  
Major Complication ◽  
Initial Step ◽  
Neurofibromatosis Type ◽  
Tumor Location ◽  
Optic Pathway ◽  
First Line ◽  
Younger Patients ◽  
Optic Pathway Gliomas

✓ The growth rate of optic pathway gliomas (OPGs) is unpredictable and quite variable, especially in children with neurofibromatosis Type 1 (NF1). Close neuroophthalmalogical clinical follow-up with serial imaging (magnetic resonance imaging of the brain with and without contrast enhancement) is the recommended initial step in management to establish the growth rate of the lesion in an individual patient. Typically, only symptomatic and/or radiographically growing tumors require treatment, and observation is the accepted first-line option. Although both chemotherapy and radiotherapy can stabilize growth or even decrease the size of tumors, chemotherapy, especially in younger patients, has fewer side effects than radiation therapy (such as secondary tumors, radiation necrosis, and Moyomoya disease) and is generally considered the first-line treatment for progressive lesions in younger patients. The tumor location defines prognosis in OPGs; optic nerve gliomas (ONG) have the lowest rate of complications and death, and optic chiasm and retrochiasmal gliomas the highest. Although the major complication of an OPG is visual loss, hypothalamic involvement can lead to death. Resection is an option for ONGs but is generally reserved for tumors confined to the optic nerve with poor or no vision, or for patients with severe, cosmetically unappealing proptosis, producing severe pain or exposure keratopathy in a blind eye. Resection is generally not an option for intrinsic chiasmal or retrochiasmal OPGs. Extrinsic (exophytic) components can be debulked surgically, and surgery can be performed for hydrocephalus (ventriculoperitoneal shunt placement). The approach to a patient with OPG must be individualized based on tumor location, radiographic or clinical progression, the presence of NF1, and a risk–benefit comparison for treatment.

Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1

2006 ◽  
Vol 36 (11) ◽  
pp. 1200-1203 ◽  
Author(s):  
Ioannis Nikas ◽  
Maria Theofanopoulou ◽  
Penelope Lampropoulou ◽  
Apostolos Pourtsidis ◽  
Christiana Hadjigeorgi ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Neurofibromatosis 1 ◽  
Optic Nerve Sheath ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Dural Ectasia ◽  
Nerve Sheath

scholarly journals C6 Cell Injection into the Optic Nerve of Long-Evans Rats: A Short-Term Model of Optic Pathway Gliomas

2020 ◽  
Vol 29 ◽  
pp. 096368972096438
Author(s):  
Deepti Singh ◽  
Pierre C. Dromel ◽  
Tatiana Perepelkina ◽  
Petr Baranov ◽  
Michael Young
Keyword(s):  
Optic Nerve ◽  
Glial Cells ◽  
Treatment Options ◽  
Genetic Defect ◽  
Tumor Formation ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Optic Pathway ◽  
Rat Glioma ◽  
Long Evans

The optic pathway glioma (OPG) is a slow-growing brain tumor that arises along the optic nerve or its downstream connections and causing vision to gradually worsen with time. This tumor forms in children with a genetic condition called neurofibromatosis type 1 (NF1), causing tumors to grow on nerves. In normal conditions, glial cells are there to support and protect nerve cells but, in NF1-OPG, glial cells have a genetic defect and grow out of control forming a tumor called a glioma. There are no rat models of NF1-OPG that can be used to explore various treatment options, and mouse models make interventional studies difficult due to their small eye size. We have created a model in which to study the progression of tumor growth in the optic nerve and establish the anatomical and functional consequences of the model and determine its suitability to serve as a surrogate for human disease. C6 rat glioma cells were injected into the optic nerve of Long-Evans rats and allowed to proliferate for 2 weeks. The eye clearly showed proptosis and lens opacity was observed, likely due to increased intraocular pressure caused by growing tumors. Hematoxylin–eosin staining showed marked cellularity, with hyperchromatism and pleomorphism. There was prominent area of necrosis with neoplastic cells palisading around the penumbra. Immunostaining with markers such as S100, β-tubulin III, Foxp3, CD45, Vimentin, and Ki67 confirmed low-grade tumor formation, with a mild immune response. Our results show the utility of a surgically induced rat model of OPG that may be used for exploring various treatment options for NF1 ocular tumors.

Optic nerve head vessel density in different stages of pseudoexfoliation disease

2020 ◽  
pp. bjophthalmol-2020-317605
Author(s):  
Mona Safizadeh ◽  
Amirreza Shaabani ◽  
Alireza Kamalipour ◽  
Masoud Aghsaei Fard ◽  
Kaileen Yeh ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Optic Nerve Head ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Capillary Density ◽  
Cross Sectional Study ◽  
Vascular Density ◽  
Cross Sectional ◽  
Sectoral Differences ◽  
Fellow Eyes

PurposeTo evaluate the superficial vascular density of the optic nerve head in different stages of pseudoexfoliation disease using optical coherence tomography angiography (OCTA).MethodsIn this cross-sectional study, 57 normal eyes, 41 eyes with pseudoexfoliation syndrome (PXS), 82 eyes with pseudoexfoliation glaucoma (PXG) and 27 non-glaucomatous fellow eyes of PXG (NL-PXG) that had OCTA were included. Circumpapillary RNFL (cpRNFL) thickness and circumpapillary capillary density (cpCD) were compared among the groups after adjusting for confounders using linear-mixed model.ResultsPXG eyes had thinner global RNFL and lower cpCD (74.2±14.3 µm and 36.7±10.0%) than control (103.3±8.6 µm and 52.5±2.3%), PXS (96.8±8.8 µm and 51.5±2.3%), and NL-PXG eyes (96.3±11.1 µm and 50.1±3.9%) (p<0.001). After adjustment for age, gender and signal strength index, global cpRNFL thickness was comparable among control, PXS and NL-PXG. NL-PXG had the lowest cpCD (p=0.045) and sectoral cpCD compared to PXS and control eyes. Although cpCD was comparable between control and PXS (p=0.425) eyes, sectoral differences (p=0.009 and 0.004, for inferonasal and temporal-inferior cpCD, respectively) were detectable between the two groups. AUROC for differentiating NL-PXG eyes from normal were better for cpCD (0.78) compared to cpRNLF (0.69).ConclusionsOCTA can detect reduced capillary density before significant changes in cpRNFL in fellow eyes of PXG patients. This can enable earlier detection of glaucomatous loss in pseudoexfoliation disease and enhance management of the disease.

Visual field fluctuations correlating with the menstrual cycle in a patient with optic pathway glioma

2020 ◽  
Vol 13 (4) ◽  
pp. e234014
Author(s):  
Adam Jacobson ◽  
Marcia Leonard ◽  
Patricia Robertson ◽  
Brenda L Bohnsack
Keyword(s):  
Optic Nerve ◽  
Menstrual Cycle ◽  
Visual Field ◽  
Vision Loss ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Visual Field Defects ◽  
Optic Pathway ◽  
Optic Nerve Glioma ◽  
Field Fluctuations

Optic pathway gliomas are commonly associated with vision loss in children. We describe an 18-year-old woman with neurofibromatosis, type 1 and an optic nerve glioma who showed reproducible visual field defects that worsened midmenstrual cycle and returned to baseline during menses. To our knowledge, this is the first reported case of visual field fluctuations in a patient with an optic nerve glioma that correlated with her menstrual cycle.

Risk of optic pathway glioma in children with neurofibromatosis type 1 and optic nerve tortuosity or nerve sheath thickening

2015 ◽  
Vol 100 (4) ◽  
pp. 510-514 ◽  
Author(s):  
Marc H Levin ◽  
Gregory T Armstrong ◽  
Julian H Broad ◽  
Robert Zimmerman ◽  
Larissa T Bilaniuk ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Nerve Sheath

scholarly journals Dural Ectasia of the Optic Nerve: A Rare Presentation in Neurofibromatosis Type I

2019 ◽  
Vol 10 (02) ◽  
pp. 349-351
Author(s):  
Minhaj Shaikh ◽  
Pushpinder Khera ◽  
Taruna Yadav ◽  
Pawan Garg
Keyword(s):  
Optic Nerve ◽  
Neurofibromatosis Type ◽  
Optic Chiasm ◽  
Optic Pathway Glioma ◽  
Optic Glioma ◽  
Type I ◽  
Optic Pathway ◽  
Rare Presentation ◽  
Dural Ectasia ◽  
Magnetic Resonance Imaging Mri

ABSTRACTNeurofibromatosis Type 1 (NF-1) is a common neurocutaneous syndrome with a characteristic spectrum of pathologies affecting the optic pathway. Optic pathway glioma and optic nerve meningioma are two such common afflictions of the optic nerve in NF-1. Dural ectasia of the optic nerve also known as optic nerve meningocele is a rare manifestation of optic nerve involvement in NF-1. Magnetic resonance imaging (MRI) is an excellent modality to accurately identify, characterize, delineate, and differentiate dural ectasia of the optic nerve from the commoner lesions such as optic glioma and meningioma in NF-1. We describe a case of a young woman with NF and a large recurrent palpebral neurofibroma. MRI evaluation of the orbits revealed extensive ectasia of the dura lining the cerebrospinal fluid sheath around all the segments of the optic nerve and around the optic chiasm.

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