Orbital Inflammation With Optic Perineuritis in Association With COVID-19

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Orbital Inflammation With Optic Perineuritis in Association With COVID-19 Marc Dinkin, MD, Eve Feinberg, MD, Cristiano Oliveira, MD, James Tsai, MD S ince the advent of the SARS-CoV-2 pandemic, a myriad of postinfectious and parainfectious neurological and ophthalmological complications have been described. We present a case of orbital inflammation with a prominent optic perineuritis causing episodes of transient vision loss coinciding with fever related to COVID-19. A 56-year-old man with a history of hypertension, hyperlipidemia, narrow angles treated with laser iridotomy, and Duane syndrome, presented on April 20, 2020, with mild fever and headaches above the right eyebrow worse with eye movements and 1 day of recurrent episodes of a cloudy white out in his right eye lasting from 3 to 15 minutes each. Neuro-ophthalmic examination revealed visual acuities of 20/30 in the right eye and 20/20 in the left eye. A transient 2+ relative afferent pupillary defect was found but later resolved with symptoms. Intraocular pressures were 12 mm Hg in the right eye and 15 mm Hg in the left eye. Anterior examination and dilated fundus examination were unremarkable. ESR, CRP, Lyme, RPR, SPEP, and a comprehensive rheumatologic workup including ANA, dsDNA, and lupus anticoagulant profile were normal, but AST was elevated at 48 U/L. Pilocarpine was started to treat the possibility of intermittent angle closure. Amaurosis fugax was suspected, and clopidogrel was started, but carotid ultrasound and CT angiography were normal and there were no strokes on MRI. However, MRI orbits revealed perineural enhancement with extension in the orbital fat of the right eye consistent with orbital inflammation and optic perineuritis (Fig. 1). There was presumptive involvement of perineural microvasculature given the episodic vision loss. A 3-week prednisone taper was prescribed starting at 50 mg per day. The frequency of episodes lowered a week later as the pain and fever receded. Antibodies against the SARS-CoV-2 virus came back positive. A repeat MRI 2 months later showed some improvement but persistent perineuritis despite clinical improvement. An examination 3 months after presentation revealed improvement of visual acuity in the right eye to 20/20, with normal visual fields, funduscopy, and optical coherence tomog- Departments of Ophthalmology (MD, EF, CO) and Neurology (MD), Weill Cornell Medical College, New York, New York; and Department of Ophthalmology (JT), New York Eye and Ear Infirmary of Mount Sinai, New York, New York. The authors report no conflicts of interest. Address correspondence to Marc Dinkin, MD, Departments of Ophthalmology and Neurology, Weill Cornell Medical College, NY Presbyterian Hospital, 1305 York Avenue, 11th Floor, New York, NY 10021; E-mail: [e-mail] e300 raphy. He remained asymptomatic 9 months after presentation without any further corticosteroid treatment. DISCUSSION We present a unique case of painful orbital inflammatory disease that presented concurrently with fever and serological evidence of exposure to SARS-CoV-2. Orbital inflammation (OI) may affect any aspect of the orbital contents, including the extraocular muscles (orbital myositis), orbital apex (cranial neuropathies), soft tissues, and optic nerve. Symptoms include proptosis, injection, chemosis, diplopia, periorbital pain worse with eye movements, and vision loss. Various infectious and rheumatological etiologies have been associated, including IgG4 disease (1). Several inflammatory conditions causing diplopia have been reported with COVID-19 infection, including isolated abducens palsy (2), Miller Fisher syndrome (2), and myasthenia gravis (3). Reports of optic nerve involvement as seen in our case are rare, with one case of myelin oligodendrocyte glycoprotein–associated optic neuritis associated with COVID-19 (4) and another case of papillophlebitis after the disease (5). In one case of isolated abducens palsy, there was anterior perineural enhancement on MRI without vision loss (2). Although 2 cases of bacterial orbital cellulitis concomitant with COVID-19 have been reported (6), to the best of our knowledge, this is the first case of nonbacterial OI associated with COVID-19. Causation by the viral infection cannot be proven, but the simultaneous onset of the visual symptoms with the fever and ongoing radiological findings for weeks after the fever subsided suggest an immune response to the novel virus. The lack of recurrent symptoms for 9 months, despite only a 3-week steroid course, also suggests a postinfectious inflammatory reaction rather than a systemic etiology. In idiopathic cases of OI in endemic regions, COVID-19 should be considered. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: M. Dinkin, E. Feinberg, C. Oliveira, and J. Tsai; b. Acquisition of data: M. Dinkin, E. Feinberg, C. Oliveira, and J. Tsai; c. Analysis and interpretation of data: M. Dinkin, E. Feinberg, C. Oliveira, and J. Tsai. Category 2: a. Drafting the manuscript: M. Dinkin, E. Feinberg, C. Oliveira, and J. Tsai; b. Revising it for intellectual content: M. Dinkin, E. Feinberg, C. Oliveira, and J. Tsai. Category 3: a. Final approval of the completed manuscript: M. Dinkin, E. Feinberg, C. Oliveira, and J. Tsai. Dinkin et al: J Neuro-Ophthalmol 2022; 42: e300-e301 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 1. Enhancement within the soft tissue of the right orbit, especially around the optic nerve, is seen on postcontrast T1 axial (A) and coronal (B) MRI (arrows). REFERENCES 1. Plaza JA, Garrity JA, Dogan A, Ananthamurthy A, Witzig TE, Salomão DR. Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease. Arch Ophthalmol. 2011;129:421–428. 2. Dinkin M, Gao V, Kahan J, Bobker S, Simonetto M, Wechsler P, Harpe J, Greer C, Mints G, Salama G, Tsiouris AJ, Leifer D. COVID-19 presenting with ophthalmoparesis from cranial nerve palsy. Neurology. 2020;95:221–223. 3. Restivo DA, Centonze D, Alesina A, Marchese-Ragona R. Myasthenia gravis associated with SARS-CoV-2 infection [published online ahead of print, 2020 Aug 10]. Ann Intern Med. 2020:L20–L0845. Dinkin et al: J Neuro-Ophthalmol 2022; 42: e300-e301 4. Zhou S, Jones-Lopez EC, Soneji DJ, Azevedo CJ, Patel VR. Myelin oligodendrocyte glycoprotein Antibody-associated optic neuritis and myelitis in COVID-19. J Neuroophthalmol. 2020;40:398–402. 5. Insausti-García A, Reche-Sainz JA, Ruiz-Arranz C, López Vázquez Á, Ferro-Osuna M. Papillophlebitis in a COVID-19 patient: inflammation and hypercoagulable state [published online ahead of print, 2020 Jul 30]. Eur J Ophthalmol. 2020. doi: 10.1177/1120672120947591. 6. Turbin RE, Wawrzusin PJ, Sakla NM, Traba CM, Wong KG, Mirani N, Eloy JA, Nimchinsky EA. Orbital cellulitis, sinusitis and intracranial abnormalities in two adolescents with COVID-19. Orbit. 2020;39:305–310. e301 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.