Atypical Case of Optic Nerve Head Edema: Painful, Enhancing and Treacherous

A 42-year-old female patient was referred to our neuro-ophthalmology clinic for unilateral gradual decrease of vision in her right eye over two weeks. She also reported light pain on extraocular movements as well as daily headaches. She did not have any focal neurological deficit on history and no fever, chills, or night sweats. She had received the second dose of her COVID-19 vaccine two days before the onset of symptoms and had a history of gonorrhea treated 2 months prior. Her visual acuity was 20/50 in the right eye and 20/20 in the left one. She did have a right relative afferent pupillary defect (RAPD), graded as 1+. Extraocular movements were full in both eyes, including normal saccades and pursuit. Color vision was decreased in her right eye with a score of 7/11 on HRR compared to 11/11 in her left eye. She did not have proptosis on Hertel examination. On slit lamp exam, her anterior segment was unremarkable, including a calm anterior chamber. She had severe right optic nerve edema graded as 5+. The disc was elevated over 360 degrees and all major vessels were obscured. She exhibited peripapillary hemorrhages as well as cotton wool spots. There was some subretinal fluid under the papillomacular bundle, but no macular star. Posterior pole and periphery showed some dot-blot and flame-shaped hemorrhages in the right eye only. No cells were visible in the vitreous. The left optic nerve was completely normal with clear margins and a cup-to-disc ratio of 0.3 Optical coherence tomography showed diffuse elevation of the retinal nerve fiber layer (rNFL) in the right eye (199 versus 98 μm). Ganglion cell complex was atrophied (54 versus 87 μm). Visual field testing (SITA 24-2) showed a cecocentral scotoma as well as inferior arcuate defect. Left eye had a complete visual field.