Acute Visual Loss Related to Retinal Vascular Occlusion Secondary to Visual Pathway Primary Glioblastoma.

M alignant gliomas are invasive tumors originating in glial cells of the central nervous system (CNS); their origin primary visual pathways gliomas are very infrequent (1). Optic gliomas can be benign or malignant tumors and represent approximately 1% of intracranial tumors and 65% of optic nerve primary tumors (2). Optic gliomas have a benign course in 90% of cases and are diagnosed in patients under 20 years of age; the most common type is pilocytic astrocytoma (WHO Grade I), which can be associated with neurofibromatosis Type 1 between 10% and 70% of cases (2). On the other hand, malignant optic nerve gliomas occur mainly in people over 50 years and have aggressive clinical course, fast growth, and very poor prognosis (3). At onset, unilateral or bilateral decreased vision occurs in 70% of patients, leading to blindness usually within 3 months and death in less than 1 year (4). Here, we present a patient that presented with painful acute visual loss secondary to malignant optic glioma. Informed consent was obtained for publication. A 61-year-old woman with a medical history of mild hypertension and hypothyroidism presented to the emergency department after 2 days of acute visual loss of the right eye, orbital pain, and right side headache. Physical examination of the right eye showed the following findings: a. decreased visual acuity limited to light perception, b. mild palpebral ptosis, c. relative afferent pupillary defect, d. painful ocular movements, e. mild vitreal hemorrhage, f. optic disc edema with exudates and peridiscal hemorrhages, and g. multiple retinal hemorrhages and vascular tortuosity (Fig. 1A). Intraocular pressure was normal in both eyes. Left eye examination was normal. The clinical presentation was interpreted as an atypical anterior optic neuritis associated with central retinal vein occlusion. A systemic workup including blood test and cerebrospinal fluid was performed, and it did not show evidence of infection, immunologic disorders, vasculitis, or metabolic abnormalities. Brain MRI showed a hyperintensity signal in T2-weighted sequences at the right optic nerve in intraorbital, intracanalicular, and prechiasmatic portions associated with contrast enhancement after gadolinium injection (Fig. 1). Moreover, brain MRI also showed a T2 hyperintensity signal at the right temporal uncus, hypocampus, and thalamus (Fig. 1B-E). An inflammatory cause was suspected because of brain involvement reported in MRI, and the patient was treated with intravenous methylprednisolone (1 g a day for 5 days), but visual acuity did not recover and she was discharged. Two weeks later, she presented with chemosis and visual deterioration. The clinical exploration right eye showed complete visual loss, optic nerve atrophy, arteriolar and vein atherosclerosis, bloodless vessels, and retinal pallor suggesting a combined central arterial and vein retinal occlusion (Fig. 1F) and eye contralateral hemianopsia. A brain MRI was performed, and it showed findings of high-grade tumor originating in the right visual pathways (Fig. 1G–J). A stereotaxic brain biopsy at the right temporal lobe was performed, and the anatomopathology analysis reported a glioblastoma (Fig. 2). The patient was treated with intensity-modulated radiation therapy 6 MV photons at planning target volume of 2 Gy/day (total dose 60 Gy) concomitant to temozolomide followed by mensual temozolomide as 5/28 days schedule. However, after 4 cycles of chemotherapy, brain MRI showed increase of tumoral size and mass effect due to tumor progression because she developed a left hemiplegia and central neuropathic thalamic pain syndrome (Fig. 3). Bevacizumab was added to treatment, but she presented a severe pulmonary embolism after 1 cycle and secondary clinical deterioration leading to chemotherapy interruption, and she died 12 months from diagnosis. Malignant gliomas (anaplastic astrocytoma, anaplastic oligodendroglioma, oligoastrocytoma, and glioblastoma according to WHO classification 2016) (3) are diffuse CNS Department of Neuro-ophthalmology (MLM-C), Universidad Pontificia Bolivariana, Medellín, Colombia; Department of Ophthalmology (YL-N), Universidad Pontificia Bolivariana, Medellín, Colombia; Department of Neurosurgery (FL-O), Hospital Pablo Tobón Uribe, Medellín, Colombia; Department of Neurosurgery (EEP-M), Universidad de Antioquia, Medellín, Colombia; and Departments of Neuroradiology (FR, LG), Radiotherapy (BP-A), Pathology (BL-M), and Department of Neuro-oncology (GAR-B), Hospital Pablo Tobón Uribe, Medellín, Colombia.