Cotton-wool spots as the initial ocular manifestation in Wegener's granulomatosis

Various ocular manifestations have been described in Wegener's granulomatosis. Orbital involvement is common and manifestations in the anterior segment of the eye such as keratitis, scleritis, and conjunctivitis may occur. Involvement of the posterior segment presenting with major vascular occlusions or anterior ischemic neuropathy is rare. This case report presents a patient with bilateral cotton-wool spots at the posterior pole associated with histologically proven Wegener's granulomatosis. This finding, as an initial ocular manifestation of the disease, is very uncommon and may be interpreted as focal retinal inflammatory vasculitis. This was supported by the rapid positive response to systemic combined immunosuppressive and steroidal treatment during a 30-month period of review. The presence of cotton-wool spots in association with systemic signs of the disease should be considered in establishing the diagnosis of Wegener's granulomatosis.