Abstract
BackgroundVisual 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.MethodsWe 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.FindingsOf 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).InterpretationOur 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.
Highlights
Optic pathway glioma (OPG) accounts for approximately 5% of brain tumors in children [1], and in approximately 30% of cases, OPG is associated with neurofibromatosis type 1 (NF1) [2,3]
What is the frequency of low vision in patients treated during childhood for OPG with up-front chemotherapy? Are there any initial or evolving factors associated with very-long-term vision loss? We aimed to study this very-long-term visual impact in a historical cohort of patients treated with up-front BB-SFOP (Baby Brain-French Society of Pediatric Oncology) chemotherapy [10]
According to the multivariate logistic regression analysis (Table 6), we found that only age < 1 year at diagnosis, the need for surgery for ICHT, and 7 tumor locations were significantly associated with a poor prognosis of long-term visual acuity (VA) (WHO 3, 4 or 5)
Summary
Optic pathway glioma (OPG) accounts for approximately 5% of brain tumors in children [1], and in approximately 30% of cases, OPG is associated with neurofibromatosis type 1 (NF1) [2,3]. Possible damage to the visual pathway and its impact on vision are the major issues of these tumors, and these possible outcomes are often a primary reason for initiating treatment. Numerous reports have aimed to assess prognostic factors for visual outcomes, few have examined the very-long-term evolution (more than 10 years after diagnosis) of visual outcome, and the conclusions of these studies are often divergent. They often differ significantly in terms of the study population (NF1 or non-NF1), treatments applied (or not applied), duration of follow-up, or criteria used to assess visual acuity (VA). We aimed to study this very-long-term visual impact in a historical cohort of patients treated with up-front BB-SFOP (Baby Brain-French Society of Pediatric Oncology) chemotherapy [10]. Editor: Roland A Ammann, University of Bern, SWITZERLAND Received: October 26, 2018 Accepted: January 28, 2019 Published: March 8, 2019
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