Bilateral isolated retrobulbar optic neuropathy in limited Wegener's granulomatosis

Wegener's granulomatosis causes a variety of ophthalmologic disorders, some of which occasionally constitute the initial presentation of the disease. We describe a patient who presented with bilateral, isolated, consecutive, posterior optic neuropathy with light perception and no light perception vision. The patient had no other symptoms or signs of orbital disease. Investigations revealed cavitary lung lesions, positive antineutrophilic cytoplasmic antibody (ANCA) titers, subtle focal enhancement of the intracanalicular optic nerves on magnetic resonance imaging, and a confirmatory bronchial biopsy. This exceedingly rare ocular presentation of Wegener's granulomatosis may pose a diagnostic quandary.