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Middle-aged Woman With Optic Neuritis and a Rapidly Progressing Retinal Vaso-occlusive Disease

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 40s presented to an outside ophthalmologist with a 3-day history of an inferior scotoma in her right eye. Her Snellen visual acuities were 20/25 OD and 20/20 OS. Her intraocular pressures were 21 mm Hg OD and 14 mm Hg OS. The right eye had a relative afferent pupillary defect and mild optic nerve edema. To rule out optic neuritis, a magnetic resonance imaging scan of the brain and orbits was obtained; the results were unremarkable. Six days after symptom onset, her visual acuity decreased to 20/70 OD and the disc edema increased. In addition, the right eye had developed 2+ anterior chamber cell, keratic precipitates, and a possible branch vein occlusion. At this time the patient was referred to our clinic. On presentation 1 day later, visual acuity was light perception OD and still 20/20 OS. The right eye had 3+ anterior chamber cell, inferior keratic precipitates, and 1+ vitreous cell. The right optic nerve had grade 4 disc edema with flame-shaped peripapillary hemorrhages. The fundus examination results of the right eye revealed the following superior quadrant retinal findings: extensive intraretinal and preretinal hemorrhages along vessels, arteriolar attenuation and sheathing, venous engorgement, and tortuosity. The inferior midperiphery had numerous small areas of deep retinal whitening (Figure 1). Examination results of the left eye were normal.

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Acute retinal necrosis

C. Administer high-dose oral valacyclovir (2 g, 3 times daily)

This patient had an acute, monocular process characterized by optic disc edema, hemorrhagic occlusions of the retinal arteries and veins, and anterior segment and vitreous inflammation. Systemic corticosteroids (choice A) or intraocular steroids (choice B) are contraindicated before an appropriate infectious workup has been completed. Intramuscular penicillin G (choice D) would not be administered without further proof of infection, and if the patient had ocular syphilis, the appropriate treatment would be intravenous, not intramuscular, penicillin. The presentation was suspicious for viral retinitis (acute retinal necrosis) and clearly rapidly progressive and vision threatening. Oral valacyclovir was administered empirically (2 g, 3 times daily; choice C); intravitreal antiviral medication was deferred pending the results of aqueous polymerase chain reaction (PCR) testing. The PCR results were positive for varicella-zoster virus, so she received intravenous (IV) acyclovir and intravitreal foscarnet.

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Article Information

Corresponding Author: Wendy M. Smith, MD, Department of Ophthalmology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (smith.wendy1@mayo.edu).

Published Online: May 28, 2020. doi:10.1001/jamaophthalmol.2020.0605

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Schoenberger  SD , Kim  SJ , Thorne  JE ,  et al.  Diagnosis and treatment of acute retinal necrosis: a report by the American Academy of Ophthalmology.   Ophthalmology. 2017;124(3):382-392. doi:10.1016/j.ophtha.2016.11.007PubMedGoogle ScholarCrossref
2.
Franco-Paredes  C , Bellehemeur  T , Merchant  A , Sanghi  P , DiazGranados  C , Rimland  D .  Aseptic meningitis and optic neuritis preceding varicella-zoster progressive outer retinal necrosis in a patient with AIDS.   AIDS. 2002;16(7):1045-1049. doi:10.1097/00002030-200205030-00011PubMedGoogle ScholarCrossref
3.
Liu  JZ , Brown  P , Tselis  A .  Unilateral retrobulbar optic neuritis due to varicella zoster virus in a patient with AIDS: a case report and review of the literature.   J Neurol Sci. 2005;237(1-2):97-101. doi:10.1016/j.jns.2005.05.005PubMedGoogle ScholarCrossref
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5.
Holland  GN .  The progressive outer retinal necrosis syndrome.   Int Ophthalmol. 1994;18(3):163-165. doi:10.1007/BF00915966PubMedGoogle ScholarCrossref
6.
Witmer  MT , Pavan  PR , Fouraker  BD , Levy-Clarke  GA .  Acute retinal necrosis associated optic neuropathy.   Acta Ophthalmol. 2011;89(7):599-607. doi:10.1111/j.1755-3768.2010.01911.xPubMedGoogle ScholarCrossref
7.
Baltinas  J , Lightman  S , Tomkins-Netzer  O .  Comparing treatment of acute retinal necrosis with either oral valacyclovir or intravenous acyclovir.   Am J Ophthalmol. 2018;188:173-180. doi:10.1016/j.ajo.2018.02.001PubMedGoogle ScholarCrossref
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