Orbital apex syndrome

A 70-year-old woman presented with a one-month history of progressive, painful loss of vision in the right eye (OD), a droopy right upper eyelid, and horizontal binocular diplopia. Past medical history was significant for hypertension, diabetes mellitus type II, and breast cancer (status post-mastectomy and -chemotherapy). Review of systems was significant for nausea, vomiting, and malaise. On examination, visual acuity was light perception OD and 20/25 in the left eye (OS). The right pupil measured 5 mm in the dark and was non-reactive to light or accommodation with a relative afferent pupillary defect (RAPD) OD (by reverse testing). The left pupil measured 4 mm in the dark and 3 mm in the light with no light-near dissociation. Ocular motility revealed complete ophthalmoplegia OD but full extraocular movements OS (Fig. 13.1). Anterior segment examination and intraocular pressure measurements were normal in both eyes (OU). External exam showed complete ptosis OD. Hertel exophthalmometry showed 3mm of proptosis OD (23mm OD compared with 20mm OS). Dilated fundus exam revealed diffuse optic disc pallor OD and a normal fundus OS. Magnetic resonance imaging (MRI) of the brain and orbits with and without contrast showed a dural-based extra-axial lesion located within the sella and extending into the right-sided cavernous sinus, superior orbital fissure, planum sphenoidale, and infundibulum (Fig. 13.2). Trans-sphenoidal biopsy revealed diffuse large B-cell lymphoma. The patient underwent staging and was treated with systemic chemotherapy and radiotherapy.