Approach Considerations
Treatment of compressive optic neuropathy should be aimed at the underlying cause. Treatment can be separated into medical management and surgery.
A favorable response to treatment with corticosteroids should not be considered as confirmation of a diagnosis until good quality MRIs and CT scans are obtained.
A practical approach for those cases in which the MRI and/or CT scan strongly indicates a meningioma (both intraorbital and intracanalicular) is to monitor the patient with serial visual acuity measurements and field testing. If visual loss progresses, consider treatment with radiation, and, if growth continues, surgery may be considered with or without embolization of feeder vessels to the tumor.
Decisions for surgical interventions to address vision loss should be made based on careful examination. Realistic expectations regarding the probability of improvement need to be discussed with the patient.
Medical Care
Corticosteroids may be useful in compressive optic neuropathy caused by inflammation (eg, sarcoid) and thyroid ophthalmopathy. Radiation also can be considered for thyroid eye disease or some anterior visual pathway tumors (eg, meningioma). [23, 24]
Surgical Care
Treatment of compressive optic neuropathy is aimed at managing the underlying cause. Consider surgical excision or decompression as a treatment option when visual failure results from optic nerve compression by a tumor. [5] Apical optic nerve tumors (eg, cavernous hemangioma) may require an orbitocranial approach. [25]
Surgical orbital decompression may be necessary for compressive optic neuropathy of thyroid eye disease.
If the tumor is intimately involved with the optic nerve, as often is the case with nerve sheath meningiomas, surgical removal often results in further loss of vision. This is thought to be due to a compromise of the shared blood supply.
If the cause of CON is trauma (eg, sheath or retrobulbar hematoma), surgical decompression may be necessary. Steroids can be used preoperatively, intraoperatively, or postoperatively. [7]
Consultations
Patients with compressive optic neuropathy should be managed in consultation with a neuro-ophthalmologist and/or a neurosurgeon, if necessary.
Activity
Prescribe polycarbonate safety glasses to patients with compressive optic neuropathy to protect the vision in the unaffected eye.
Smoking cessation in patients with thyroid ophthalmopathy may improve disease and may slow the development of CON, diplopia, and proptosis. [26]
Complications
Surgery to remove orbital tumors compressing the optic nerve is frequently associated with injury to the surrounding structures, including third, fourth, and/or sixth cranial nerves, which may result in paralytic strabismus and ptosis.
Surgery to remove lesions that are intimately involved with the nerve sheath (eg, meningiomas, schwannomas) often results in further loss of vision or blindness, and primary radiation therapy may be indicated.
Prevention
Preventive measures vary by etiology.
Long-Term Monitoring
Long-term monitoring is indicated for patients who are found to have chronic CON. Patients should seek regular care with their ophthalmologist, as well as the appropriate specialist.
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Axial MRI taken 3 weeks after the onset of distorted vision in the right eye; visual acuity is reduced to counting fingers at 1 ft. Evidence of optic nerve compression is not seen; disease in the sphenoid sinus is reported.
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MRI of same patient as in the image above taken 4 months later. Patient responded well to IV Solu-Medrol, but symptoms returned when steroids were reduced. Large mass compressing the right optic nerve is seen. Biopsy revealed lymphoma.
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A 72-year-old man with a moderate decrease in vision in the left eye (20/20 right, 20/25 left). Fundus examination revealed a normal right optic nerve.
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Same patient as in image above of a 72-year-old man with a moderate decrease in vision in the left eye (20/20 right, 20/25 left). Fundus examination revealed an atrophic left optic nerve.
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Neuroimaging study (MRI of brain and orbits) revealed an extensive meningioma involving the left orbital apex (arrow).
