• Ben Shofty, Liat Ben-Sira, Anat Kesler, George Jallo, Mari L Groves, Rajiv R Iyer, Alvaro Lassaletta, Uri Tabori, Eric Bouffet, Ulrich-Wilhelm Thomale, Hernáiz Driever Pablo P 7Pediatric Neurosurgery, Charité Universitätsmedizin, Campus Virchow Klinikum, Berlin, Germany., Shlomi Constantini, and Isolated Optic Nerve Abnormalities (IONA) Collaboration.
• 1The Gilbert Israeli Neurofibromatosis Center, Dana Children's Hospital.
• J Neurosurg Pediatr. 2017 Dec 1; 20 (6): 549-555.

AbstractOBJECTIVE Isolated optic nerve gliomas (IONGs) constitute a rare subgroup of optic pathway gliomas (OPGs). Due to the rarity of this condition and the difficulty in differentiating IONGs from other types of OPGs in most clinical series, little is known about these tumors. Currently, due to lack of evidence, they are managed the same as any other OPG. METHODS The authors conducted a multicenter retrospective cohort study aimed at determining the natural history of IONGs. Included were patients with clear-cut glioma of the optic nerve without posterior (chiasmatic/hypothalamic) involvement. At least 1 year of follow-up, 2 MRI studies, and 2 neuro-ophthalmological examinations were required for inclusion. RESULTS Thirty-six patients with 39 tumors were included in this study. Age at diagnosis ranged between 6 months and 16 years (average 6 years). The mean follow-up time was 5.6 years. Twenty-five patients had neurofibromatosis Type 1. During the follow-up period, 59% of the tumors progressed, 23% remained stable, and 18% (all with neurofibromatosis Type 1) displayed some degree of spontaneous regression. Fifty-one percent of the patients presented with visual decline, of whom 90% experienced further deterioration. Nine patients were treated with chemotherapy, 5 of whom improved visually. Ten patients underwent operation, and no local or distal recurrence was noted. CONCLUSIONS Isolated optic nerve gliomas are highly dynamic tumors. Radiological progression and visual deterioration occur in greater percentages than in the general population of patients with OPGs. Response to chemotherapy may be better in this group, and its use should be considered early in the course of the disease.

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