RESUMEN
Optic nerve is most important cranial nerve responsible for visual functioning. Optic nerve involvement leading to blindness can be seen in various diseases, which cause swelling, inflammation, ischemia of the optic nerve. The optic nerve glioma (ONG) comprises 5% of all pediatric intracranial tumor and are a leading cause of unilateral proptosis. ONGs can be associated with neurofibromatosis and are more common than meningiomas. 20-30% of ONGs become symptomatic before the age of 10 years. Visual evoked potential testing is helpful in detecting asymptomatic gliomas. Early detection and prompt management can prevent blindness from gliomas. Treatment of ONG should be tailored to the individual patient. Our patient also showed the presence of unilateral ONG with proptosis. Magnetic resonance imaging was diagnostic. Partial optic atrophy was also present.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Preescolar , Potenciales Evocados Visuales/etiología , Exoftalmia/diagnóstico , Humanos , Masculino , Neurofibromatosis/diagnóstico , Glioma del Nervio Óptico/complicaciones , Glioma del Nervio Óptico/diagnósticoRESUMEN
A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed.