Purpose of review: Varicella zoster virus (VZV) ocular infection can manifest purely as a vasculopathy that leads to retinal arteriole occlusion, without any retinitis or vasculitis. This review summarizes our current knowledge of such VZV ocular infection phenotype, incorporating initial descriptions from 1988. We describe the pathogenesis and VZV's manifestations in the retina using fundus photography, fundus fluorescein angiography, optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). Laboratory investigations, diagnostic procedures, prognoses, and treatment options are also being reviewed.
Recent findings: Ten case reports where VZV retinal vasculopathy was the primary feature observed after varicella or zoster rash are described. The retinal arteriole, cilioretinal artery, branches of retinal artery, central retinal artery and ophthalmic artery were found to be areas of more rarely affected, neither in the form of vasculitis nor retinitis. Diagnosis is typically made from positive polymerase chain reaction (PCR) for VZV from extracted intraocular fluid or positive serum or cerebrospinal fluid (CSF) anti-VZV immunoglobulin G antibody in the context of compatible ocular findings. In addition, retinal vasculopathy occurring in the setting of confirmed varicella or zoster rashes could be considered potentially pathognomonic. Pathological concepts, including direct VZV infection of affected tissue, persistent inflammation, and/or virus-induced hypercoagulability are also discussed.
Summary: VZV may produce a wide spectrum of ocular manifestations with isolated VZV retinal vasculopathy being a rarer presentation. A prompt diagnosis followed by an early treatment of systemic acyclovir with or without corticosteroids is the mainstay of treatment.
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