Screening and Diagnosis of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1 by Using Sweep Visual Evoked Potentials

Chang, Benjamin; Mirabella, Giovanni; Yagev, Ronit; Banh, Michael; Mezer, Eedy; Parkin, Patricia C.; Westall, Carol A.; Buncic, J. Raymond

doi:10.1167/iovs.06-0429

Cited by 30 publications

(25 citation statements)

References 37 publications

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“…21,22 Another limitation of serial VEPs in the screening and surveillance for OPGs is the difficulty in interpreting small changes in amplitude during follow-up. [21][22][23] In our study, RNFL assessment by OCT reached sensitivity and specificity values similar to those reported for VEP, without any problem of interpretation and allowing automatic follow-up quantification of RNFL changes. 4,24 Although MRI scanning of the brain remains the gold standard diagnostic test for OPGs, its use remains controversial in the absence of clinical signs such as decreased vision.…”

Section: Discussionsupporting

confidence: 85%

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Optical Coherence Tomography in the Diagnosis of Optic Pathway Gliomas

Parrozzani

Clementi

Kotsafti

et al. 2013

Invest. Ophthalmol. Vis. Sci.

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PURPOSE. To compare visual function assessment, optic disc evaluation by indirect ophthalmoscopy, and retinal nerve fiber layer analysis by optical coherence tomography (OCT) for the screening of optic pathway gliomas in pediatric patients (2-15 years old) affected by neurofibromatosis type 1.METHODS. Fifty-seven consecutive patients with neurofibromatosis type 1 with recent (<6 months) orbital/brain magnetic resonance images (MRI) were included. Patients underwent visual function assessment (Hyvarinen symbols chart and/or Snellen charts) and optic disc evaluation by indirect ophthalmoscopy performed by experienced, masked pediatric ophthalmologists. Spectral domain OCT was performed to assess retinal nerve fiber layer. RESULTS.Fifteen of 57 enrolled patients (26%) were affected by MRI-proven optic pathway gliomas. Visual function assessment, optic disc evaluation, and retinal nerve fiber layer analysis by OCT were feasible in 84%, 95%, and 88% of patients, respectively. Visual function assessment, retinal nerve fiber layer analysis, and optic disc evaluation results correlated with the presence of optic pathway gliomas (P ¼ 0.007, P < 0.0001, and P ¼ 0.03, respectively). Specificity and negative predictive value of each test were statistically significant in detecting optic pathway glioma (P < 0.0001), whereas only retinal nerve fiber layers analysis reached statistically significant sensitivity and positive predictive value (P ¼ 0.0386). CONCLUSIONS.Retinal nerve fiber layer analysis assessment using spectral domain OCT is superior to visual function assessment and optic disc evaluation as a clinical screening tool for optic pathway gliomas.

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Section: Discussionsupporting

confidence: 85%

“…[21][22][23] Unfortunately, VEP testing relies on experienced electrophysiologists, who are scarcely available. 21,22 Another limitation of serial VEPs in the screening and surveillance for OPGs is the difficulty in interpreting small changes in amplitude during follow-up.…”

Section: Discussionmentioning

confidence: 99%

Optical Coherence Tomography in the Diagnosis of Optic Pathway Gliomas

Parrozzani

Clementi

Kotsafti

et al. 2013

Invest. Ophthalmol. Vis. Sci.

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“…Visual evoked potentials (VEPs) are an electrophysiologic test believed to provide a functional measure of visual pathway integrity. Although VEP testing may detect OPG with some sensitivity, [42][43][44][45][46] some patients with NF1 have abnormal VEP testing despite no evidence of glioma. 47 Of greater concern is the poor diagnostic sensitivity of VEP for VA loss 48 and the poor correlation of VEP changes over time with VA changes and response to treatment.…”

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confidence: 99%

Functional outcome measures for NF1-associated optic pathway glioma clinical trials

et al. 2013

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Objective: The goal of the Response Evaluation in Neurofibromatosis and Schwannomatosis Visual Outcomes Committee is to define the best functional outcome measures for future neurofibromatosis type 1 (NF1)-associated optic pathway glioma (OPG) clinical trials. Methods:The committee considered the components of vision, other ophthalmologic parameters affected by OPG, potential biomarkers of visual function, and quality of life measures to arrive at consensus-based, evidence-driven recommendations for objective and measurable functional endpoints for OPG trials.Results: Visual acuity (VA) assessments using consistent quantitative testing methods are recommended as the main functional outcome measure for NF1-OPG clinical trials. Teller acuity cards are recommended for use as the primary VA endpoint, and HOTV as a secondary endpoint once subjects are old enough to complete it. The optic disc should be assessed for pallor, as this appears to be a contributory variable that may affect the interpretation of VA change over time. Given the importance of capturing patient-reported outcomes in clinical trials, evaluating visual quality of life using the Children's Visual Function Questionnaire as a secondary endpoint is also proposed. Conclusions:The use of these key functional endpoints will be essential for evaluating the efficacy of future OPG clinical trials. Neurology ® 2013;81 (Suppl 1):S15-S24 GLOSSARY CVFQ 5 Children's Visual Function Questionnaire; logMAR 5 logarithm of the minimum angle of resolution; MS 5 multiple sclerosis; NF1 5 neurofibromatosis type 1; OCT 5 optical coherence tomography; OPG 5 optic pathway glioma; PFS 5 progression-free survival; QOL 5 quality of life; REiNS 5 Response Evaluation in Neurofibromatosis and Schwannomatosis; RNFL 5 retinal nerve fiber layer; TAC 5 Teller acuity cards; VA 5 visual acuity; VEP 5 visual evoked potential; VF 5 visual field.Optic pathway gliomas (OPG) arise in 15%-20% of children with neurofibromatosis type 1 (NF1), occur preferentially in young children compared with adolescents or adults, and cause vision loss in as many as half of those affected.1 In this regard, the main objective in clinical management of these tumors is preservation of visual function. Although prognostic factors have been identified, there are currently no reliable indicators of future visual loss. This absence of prognostic signs has led clinicians to avoid initiating treatment until visual function has declined. When treatment is indicated, NF1-OPG are typically managed with a combination of carboplatin and vincristine-an approach that has not changed in 15 years. 2To date, OPG clinical trials have focused on imaging outcomes, with tumor response and/or progression-free survival used as measures of treatment success.

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“…Of these 69 children, 16 In 20 cases, behavioral and VEP acuity results were differed; of these, 14 gave better acuity estimates for behavioral testing (predominately primary ocular abnormality) and 6 gave better acuity estimates for VEP testing (1 non-organic loss, the rest primarily cranial disorder). One of this latter group gave no response to Teller Acuity Cards, while giving a response to a 15-min check size on VEP testing.…”

Section: Resultsmentioning

confidence: 99%

Paradoxical robust visual evoked potentials in young patients with cortical blindness

et al. 2009

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The objective of this study was to review retrospectively cases of clinically blind children in whom robust pattern visual evoked potentials (VEPs) were recorded. VEP records from a 10-year period (1990-2000) were reviewed. We searched for charts of children who were clinically cortically blind, but in whom assessment of visual acuity, using visual evoked potentials (VEPs), was normal or close to normal. The majority (77.5%) of VEP and behavioral acuity measures were concordant (subset analysis). Of the 1,113 VEP records, 9 cases (<1% of records reviewed) had clinically compromised vision with fair to good levels of visual function using VEPs. The commonality among the cases was the presence of suspected cortical visual impairment with seizures and developmental delay. VEP acuity cannot be correlated unequivocally with visually guided behaviour. In specific cases, particularly cases with developmental delay and neuroradiographic abnormalities, a child who is behaviorally blind with no clinical evidence of vision may show robust VEPs even to small patterns. This finding might be consistent with a defect of the visual association cortex.

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Screening and Diagnosis of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1 by Using Sweep Visual Evoked Potentials

Cited by 30 publications

References 37 publications

Optical Coherence Tomography in the Diagnosis of Optic Pathway Gliomas

Optical Coherence Tomography in the Diagnosis of Optic Pathway Gliomas

Functional outcome measures for NF1-associated optic pathway glioma clinical trials

Paradoxical robust visual evoked potentials in young patients with cortical blindness

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