Clinical Presentation and Outcome of Patients with Optic Pathway Glioma

Publication date: Available online 6 July 2017
Source:Pediatric Neurology
Author(s): Viviane Robert-Boire, Lorena Rosca, Yvan Samson, Luis H Ospina, Sébastien Perreault
Background: Optic pathway gliomas (OPGs) occur sporadically or in patients with neurofibromatosis type 1 (NF1). The purpose of this study is to evaluate the clinical presentation at diagnosis and at progression of patients with OPGs. Methods: We conducted a chart review of patients with OPGs diagnosed in a single center over a period of 15 years. Demographic data including age, sex, NF1 status, clinical presentation and outcome were collected. Results: Forty patients were retrieved. Of those, 23 patients had sporadic tumors (57.5 %) and 17 had NF1 related tumors (42.5 %). Among the children with NF1, there was a significant overrepresentation of females (82.3 %) (P=0.02), while among the children without NF1, there were slightly more males (56.5 %) than females (43.5 %). The presence of nystagmus was strongly associated with sporadic OPGs. Poor visual outcome was related to tumor affecting both optic pathways, hydrocephalus at diagnosis and optic nerve atrophy. Of 40 patients, 5 died of OPG complications (12.5 %) and all had sporadic tumors. Conclusion: Our study is one of the largest cohorts of OPGs with detailed description of clinical presentation at diagnosis and progression. We observed a significant difference between sporadic and NF1 OPGs in terms of demographics, clinical presentation and outcome.



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