Wegener's granulomatosis of the orbit

Abstract

To present a patient with an orbital mass and compressive optic neuropathy developed secondary to Wegener's granulomatosis (WG). The medical chart of the patient was reviewed. The clinical and laboratory findings were recorded. A 60-year-old male patient presented with complaints of bulging of his left eye, decrease in vision, and diplopia. The patient had a history of limited form of WG and had been receiving systemic chemotherapy (corticosteroids and azathioprine) for 5 years prior to his presentation. On examination, proptosis, total restrictive ophthalmoplegia and compressive optic neuropathy were found. Erythrocyte sedimentation rate increased, and c-ANCA was negative and p-ANCA was positive. Radiological examination showed a soft tissue mass in the inferior and medial orbit, extending to the apex of the orbit. An incisional orbital biopsy revealed histological findings consistent with WG. A subtotal mass excision was performed through transconjunctival orbitotomy, since the orbital findings deteriorated despite the medical treatment with cyclophosphamide and corticosteroids. After surgery, proptosis and ocular surface findings improved considerably, but ophthalmoplegia and visual loss secondary to optic neuropathy persisted. The residual orbital mass did not regrow during the follow-up of 22 months. Orbital masses due to WG may cause compressive optic neuropathy and ophthalmoplegia. If the orbital symptoms deteriorate despite the systemic immunosuppressive treatment, surgical debulking of the orbital mass may provide significant clinical improvement.