Optic Glioma with Characteristic Bilateral Optic Atrophy in a 3-Year-Old Girl

Abstract

We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. Neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N₇₅ in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right fronto-temporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. Intracranial pressure (ICP) increased to 40 cm H₂O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.

Astrocitoma difuso das vias ópticas: Relato de caso

“…Gliomas ópticos são achados freqüentes em pacientes portadores de neurofibromatose 7,30 . Entre esses gliomas, o astrocitoma tem sido muito encontrado 8,18,24,25 . Em alguns casos de neurofibromatose, os astrocitomas se apresentam em focos múltiplos 15,30 .…”

Glioma de nervo óptico: achados neuro-oftalmológicos em paciente com neurofibromatose tipo I

“…A associação de neurofibromatose e gliomas das vias ópticas geralmente ocorre em crianças jovens, sendo incomum o crescimento do tumor e o aparecimento de sintomas após os 10 anos de idade (10) . Em alguns casos de neurofibromatose, os astrocitomas se apresentam em múltiplos focos (11) . O glioma de nervo óptico faz diagnóstico diferencial com as patologias: pseudotumor inflamatório, miosite pós-infecciosa (12) , rabdomiossarcoma entre outros tumores orbitários.…”

“…Os gliomas múltiplos podem se localizar tanto em região supratentorial como infratentorial (11,13) . Bilaniuk (14) descreveu casos de neurofibromatose com gliomas múltiplos nas vias ópticas e tronco encefálico.…”

Direct Infiltration of Brainstem Glioma Along the Cranial Nerves

“…Chiasmatic pilocytic astrocytoma presenting with diencephalic syndrome, multicentric gliomas involving the optic chiasm, anaplastic astrocytomas invading the optic pathway, optic gliomas presenting with optic atrophy, and optic gliomas infiltrating into somatostatinergic pathways resulting in gigantism are but a few of the unusual ways that gliomas can manifest in addition to their well-known association with neurofibromatosis I. [6][7][8][9] There are a handful of case reports available that describe glioblastoma multiforme of the oculomotor nerve and a primary ependymoma of a cranial nerve. 4,10 In conclusion, spread of high-grade glioma, medulloblastoma, ependymoma, and germinoma along the cranial nerves through the CSF pathway is well known, but direct extension of a glioma along the cranial nerves is an unusual phenomenon.…”