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Neurofibromatosis type 1 associated optic pathway glioma in children - a follow up of 10 years or more

Kinori, M.; Armarnik, S.; Listernick, R.; Charrow, J.; Zeid, J. L.

Am J Ophthalmol. 2020 Apr 14


INTRODUCTION: Optic pathway gliomas (OPGs) are a common manifestation of Neurofibromatosis type 1 (NF1) and can cause significant visual morbidity. Very long-term follow-up of children with NF1-associated OPG's has not been reported previously. DESIGN: Retrospective observational case series. METHODS: This study included children with a documented follow-up of at least 10 years. Three final outcomes were evaluated: visual acuity (VA) per eye (i.e. in the more severely affected eye), VA per patient (i.e. VA when both eyes are open), and presence of optic nerve head pallor. RESULTS: 45 children were included with a mean follow-up time of 14 years (range 10-21). At the end of follow-up, abnormal VA (considered as moderate to severe impairment) in the more severely affected eye was present in 36% of the patients and in both eyes in 11%. Optic nerve head pallor of one or both nerves was present in 62%. In a multivariate analysis, only initial VA and optic nerve head appearance at presentation were found to predict the final outcomes. All patients but one who were asymptomatic at presentation and had normal VA and normal appearing nerves preserved their good vision in both eyes. Only one patient who had normal VA and normal appearing nerves at presentation had moderate to severe VA loss at long term follow-up. CONCLUSION: In this study, children with NF1-associated OPG who had a normal initial exam had excellent very long-term visual and anatomical outcomes. VA and optic nerve head appearance at presentation predict long term outcome.

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