Dataset Information

Long-term visual acuity in patients with optic pathway glioma treated during childhood with up-front BB-SFOP chemotherapy-Analysis of a French pediatric historical cohort.

ABSTRACT: BACKGROUND:Visual outcome is one of the main issues in the treatment of optic pathway glioma in childhood. Although the prognostic factors of low vision have been discussed extensively, no reliable indicators for visual loss exist. Therefore, we aimed to define initial and evolving factors associated with long-term vision loss. METHODS:We conducted a multicenter historical cohort study of children treated in France with up-front BB-SFOP chemotherapy between 1990 and 2004. Visual acuity performed at the long-term follow-up visit or within 6 months prior was analyzed. Logistic regression analysis was used to estimate the effects of clinical and radiological factors on long-term visual outcome. FINDINGS:Of the 180 patients in the cohort, long-term visual acuity data were available for 132 (73.3%) patients (median follow-up: 14.2 years; range: 6.1-25.6). At the last follow-up, 61/132 patients (46.2%) had impaired vision, and 35 of these patients (57.3%) were partially sighted or blind. Multivariate analysis showed that factors associated with a worse prognosis for long-term visual acuity were an age at diagnosis of < 1 year (OR 3.5 [95% CI: 1.1-11.2], p = 0.04), tumor extent (OR 4.7 [95% CI: 1.2-19.9], p = 0.03), intracranial hypertension requiring one or more surgical procedures (OR 5.6 [95% CI: 1.8-18.4], p = 0.003), and the need for additional treatment after initial BB-SFOP chemotherapy (OR 3.5 [95% CI: 1.1-11.9], p = 0.04). NF1 status did not appear as a prognostic factor, but in non-NF1 patients, a decrease in tumor volume with contrast enhancement after BB-SFOP chemotherapy was directly associated with a better visual prognosis (OR 0.8 [95% CI: 0.8-0.9], p = 0.04). INTERPRETATION:Our study confirms that a large proportion of children with optic pathway glioma have poor long-term outcomes of visual acuity. These data suggest new prognostic factors for visual acuity, but these results need to be confirmed further by large- and international-scale studies.

SUBMITTER: Rakotonjanahary J

PROVIDER: S-EPMC6407847 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Long-term visual acuity in patients with optic pathway glioma treated during childhood with up-front BB-SFOP chemotherapy-Analysis of a French pediatric historical cohort.

Rakotonjanahary Josué J Gravier Nicolas N Lambron Julien J De Carli Emilie E Toulgoat Frédérique F Delion Matthieu M Pellier Isabelle I Rialland Xavier X

PloS one 20190308 3

<h4>Background</h4>Visual outcome is one of the main issues in the treatment of optic pathway glioma in childhood. Although the prognostic factors of low vision have been discussed extensively, no reliable indicators for visual loss exist. Therefore, we aimed to define initial and evolving factors associated with long-term vision loss.<h4>Methods</h4>We conducted a multicenter historical cohort study of children treated in France with up-front BB-SFOP chemotherapy between 1990 and 2004. Visual a ...[more]

PMID: 30849081

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Project description:Optic neuritis (ON) has been considered to be an important factor in the diagnosis of multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD), making ON detection increasingly critical for early diagnosis. Furthermore, subclinical ONs presenting no distinct decrease in visual acuity can be missed. Low contrast visual acuity (LC-VA) is known to be able to capture visual loss not seen in conventional high-contrast visual acuity (HC-VA) in MS. Therefore, to increase the sensitivity of ON detection, we investigated the advantage of LC-VA over conventional HC-VA. One hundred and eight patients with demyelinating disease (35 MS, 73 NMOSD) with ON at least 3 months prior and 35 controls underwent neuro-ophthalmic evaluation, including best-corrected conventional high contrast visual acuity (HC-VA) and 2.5% and 1.25% low contrast visual acuity (LC-VA). Receiver operating characteristic (ROC) curve analysis and the area under the curve (AUC) of various visual functions were used to determine the most relevant visual function test for the detection of optic nerve involvement. Additionally, the optimal cutoff point was obtained from the Youden index (J-index) as the points with the best sensitivity-specificity balance. When distinguishing ON from non-ON, the area under the ROC curve (AUC) was highest for the 2.5% LC-VA (0.835, P < 0.001; sensitivity 71.5%, specificity 88.6%), while it was 0.710 (P < 0.001) for the HC-VA and 0.770 (P < 0.001) for the 1.25% LC-VA. In discriminating between controls and ON, the AUC was also highest for the 2.5% LC-VA 0.754 (P < 0.001; sensitivity 71.5%, specificity 78.5%), while it was 0.719 (P < 0.001) for HC-VA and 0.688 (P < 0.001) for 1.25% LC-VA. In eyes with a history of ON (n = 137), the HC-VA and 2.5% LC-VA were abnormal in 64.2 and 71.5%, respectively (P < 0.001), with their combination detecting abnormalities in approximately 85.4% (P < 0.001). The 2.5% LC-VA was superior to HC-VA in detecting ON when distinguishing ON from non-ON or control. The 2.5% LC-VA might be a useful, feasible, and rapid method to detect ON. Furthermore, combining 2.5% LC-VA with conventional HC-VA would be better for detecting optic nerve involvements.

Project description:ImportanceOptic neuritis (ON) in children is uncommon. There are limited prospective data for visual acuity (VA) outcomes, associated diseases, and neuroimaging findings. Prospective data from a large sample would be useful for counseling families on treatment decisions and prognosis.ObjectiveTo prospectively study children with a first episode of ON, describe VA after 6 months, and ascertain the network's (Pediatric Eye Disease Investigator Group and Neuro-Ophthalmology Research Disease Investigator Consortium) ability to enroll pediatric patients with ON prospectively.Design, setting, and participantsThis nonrandomized cohort study was conducted from September 20, 2016, to July 20, 2018, at 23 sites in the United States and Canada in pediatric ophthalmology or neuro-ophthalmology clinics. A total of 44 children (aged 3-15 years) presented with a first episode of ON (visual loss, pain on eye movements, or both) within 2 weeks of symptom onset and at least 1 of the following in the affected eye: a distance high-contrast VA (HCVA) deficit of at least 0.2 logMAR below age-based norms, diminished color vision, abnormal visual field, or optic disc swelling. Exclusion criteria included preexisting ocular abnormalities or a previous episode of ON.Main outcomes and measuresPrimary outcomes were monocular HCVA and low-contrast VA at 6 months. Secondary outcomes were neuroimaging, associated diagnoses, and antibodies for neuromyelitis optica and myelin oligodendrocyte glycoprotein.ResultsA total of 44 children (mean age [SD], 10.2 [3.5] years; 26 boys [59%]; 23 White individuals [52%]; 54 eyes) were enrolled in the study. Sixteen patients (36%) had bilateral ON. Magnetic resonance imaging revealed white matter lesions in 23 children (52%). Of these children, 8 had myelin oligodendrocyte glycoprotein-associated demyelination (18%), 7 had acute disseminated encephalomyelitis (16%), 5 had multiple sclerosis (11%), and 3 had neuromyelitis optica (7%). The baseline mean HCVA was 0.95 logMAR (20/200), which improved by a mean 0.76 logMAR (95% CI, 0.54-0.99; range, -0.70 to 1.80) to 0.12 logMAR (20/25) at 6 months. The baseline mean distance low-contrast VA was 1.49 logMAR (20/640) and improved by a mean 0.72 logMAR (95% CI, 0.54-0.89; range, -0.20 to 1.50) to 0.73 logMAR (20/100) at 6 months. Baseline HCVA was worse in younger participants (aged <10 years) with associated neurologic autoimmune diagnoses, white matter lesions, and in those of non-White race and non-Hispanic ethnicity. The data did not suggest a statistically significant association between baseline factors and improvement in HCVA.Conclusions and relevanceThe study network did not reach its targeted enrollment of 100 pediatric patients with ON over 2 years. This indicates that future treatment trials may need to use different inclusion criteria or plan a longer enrollment period to account for the rarity of the disease. Despite poor VA at presentation, most children had marked improvement by 6 months. Associated neurologic autoimmune diagnoses were common. These findings can be used to counsel families about the disease.

Dataset Information

Long-term visual acuity in patients with optic pathway glioma treated during childhood with up-front BB-SFOP chemotherapy-Analysis of a French pediatric historical cohort.

Publications

Long-term visual acuity in patients with optic pathway glioma treated during childhood with up-front BB-SFOP chemotherapy-Analysis of a French pediatric historical cohort.

Similar Datasets

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