Primary Wegener’s Granulomatosis of the Orbital Apex with Initial Optic Nerve Infiltration

Abstract

Wegener's granulomatosis can involve the orbit and sometimes the optic nerve. This usually occurs as a result of contiguous spread from affected sinuses or extraocular muscles, or from a vasculitic posterior optic neuropathy. However, we present an unusual case of isolated orbital apex infiltrative disease in a patient with known Wegener's granulomatosis. This initially caused painless optic neuropathy and progressed to painful ophthalmoplegia and blindness. Optic nerve biopsy, performed to exclude methotrexate-induced lymphoma, confirmed optic nerve infiltration. The condition was refractory to high-dose pulsed methylprednisolone but the patient gained symptomatic relief from rituximab. Wegener's granulomatosis should be considered in cases of isolated posterior optic neuropathy, and close attention should be placed on imaging the orbital apex.

Wegener Granulomatosis-associated Optic Perineural Hypertrophy and Optic Neuropathy

“…The radiological hallmarks of the present patient did not reveal those findings. Previous reports advocated fibronecrosis and gadolinium-enhancing lesions in the optic nerve sheet (1,2). Orbital MRI of our patient highlighted another possibility that hypertrophic pachymeningitisassociated optic perineural hypertrophy could cause superficial damage of the optic nerve in WG.…”

Low dose radiotherapy as an effective treatment in a patient with solitary Wegener’s granulomatosis resistant to systemic treatment –

“…It should be stressed that any organ may be affected during disease progression [9, 10]. Wegener's granulomatosis is characterized by a biphasic course: initially the disease is limited to the respiratory tract (upper and lower), then generalization and occurrence of different symptoms resulting from visceral organs’ inclusion appear.…”

Pain in Optic Perineuritis: Author Response

“…[3][4][5][6][7] To set the record right, in our case very mild pain, reported as ''discomfort,'' was present. 3 We do, however, suggest that the presence of such mild pain not worsening on eye movements did not conclusively permit distinguishing the optic perineuritis from the coexisting sinusitis in our case.…”