July 15, 2021
Authored by Burton Wisotsky, MD
A 15 year old boy was referred for optic neuropathy in his left eye. Past ocular and medical history are unremarkable. The patient noted reduced vision in the left eye over the past several months. He assumed it was due to increasing myopia. He was noted to have an optic nerve issue and was referred for examination.
On examination, VA was OD: 20/20 and OS: 20/200. IOP’s were normal. The anterior segments were normal. There was a 3+ RAPD OS. Visual fields are noted below:
DFE of the right eye was normal. There was no evidence of optic nerve or retinal disease. In the left eye the optic nerve was markedly swollen and somewhat hyperemic. The retina was unremarkable:
The right eye has a myopic nerve tilt – the left optic nerve is markedly swollen. Differential diagnosis in this age group includes demyelinating optic neuropathy, inflammatory optic neuropathy, asymmetric papilledema from increased intracranial pressure from brain tumor or pediatric pseudotumor, optic nerve infiltration, or optic nerve tumor. We scheduled an urgent MRI. The MRI was consistent with optic nerve glioma.
Optic nerve gliomas are most common in children. They are often associated with Neurofibromatosis type I. They are considered benign tumors although local growth can cause optic nerve damage. In a worst case scenario the tumor can continue to grow into the brain and cause other intracranial issues. The tumor since it is slow growing responds poorly to radiation. It can occasionally respond to chemotherapy. Surgery is indicated if it can be resected or debulked safely. Gliomas in adults have a different course. Most are malignant with a poor prognosis. Our patient had no evidence of Neurofibromatosis. His neurosurgical consultation advised observation for the time being to see if the tumor showed evidence of growth. Although this will likely not be a life threatening tumor, it will certainly have devastating visual implications for his left eye.
Please note MRI image showing oblong optic nerve glioma indenting back of globe and extending beyond orbital apex.
On further examination by a Neurofibromatosis specialist, a faint cafe au lait spot was detected on the patient’s back. He was thus officially diagnosed with Neurofibromatosis.