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Abstract
A case of bilateral subacute retrobulbar progressive and steroid-responsive optic neuropathy is presented, in which paranasal sinus biopsy revealed Wegener's granulomatosis. Clinical or radiological signs of orbital involvement were absent and the response to cyclophosphamide was satisfactory. The authors discuss various pathogenetic mechanisms of optic nerve involvement in this disease, the more common modalities being compression by intraorbital granuloma and anterior ischemic optic neuropathy. Vasculitis affecting the small vessels at the posterior portion of the optic nerve is the proposed mechanism in our case.