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Review

Varicella zoster retinal vasculitis without retinitis: a literature review

ORCID Icon, , ORCID Icon, ORCID Icon & ORCID Icon
Pages 333-341 | Received 28 Jul 2022, Accepted 14 Oct 2022, Published online: 26 Oct 2022
 

ABSTRACT

Introduction

Varicella zoster virus (VZV) uveitis presenting as acute retinal necrosis with vasculitis is well known, but VZV-vasculitis without retinitis is rarely reported. Identification of such presentation can be challenging, especially when other signs of ocular VZV are absent. This is particularly important before considering immunomodulatory therapy for retinal vasculitis without systemic manifestations.

Areas covered

An online English language literature search for ‘VZV retinal vasculitis presenting without retinitis’ was made and case reports and case series published from 1995 to 2020 were reviewed. The search revealed 26 cases from 21 reports in 25 years. The clinical manifestation, morphology of vasculitis, visual and treatment outcomes were studied.

Expert opinion

Recent or old history of Herpes Zoster Ophthalmicus or chickenpox especially in young individuals should be elicited in isolated retinal vasculitis. Diagnostic relevance of PCR and serological tests should be investigated further in larger studies. Occlusive nature of vasculitis, including arteriolitis, is common in VZV infection. In chronic cases, end point of antiviral therapy remains unknown. Patients should be warned of grave visual prognosis despite adequate treatment. The importance of adjuvant anti-thrombotic therapy along with anti-viral and immunomodulatory therapy needs to be explored further.

Article highlights

  • Varicella zoster retinal vasculitis (VRV) can present without evidence of retinitis. When anterior segment and systemic clues are absent, the diagnosis is challenging.

  • Younger individuals can be affected, and a history of chicken pox is frequently present in such cases. Right eye involvement is commonly observed.

  • Hemorrhagic or occlusive vasculitis especially arteriolitis, and rarely frosted branch angiitis can be observed in VRV. Inflammatory signs such as vascular sheathing and vitritis can remain absent.

  • In the absence of other pathognomonic signs, analysis of ocular fluid for polymerase chain reaction (PCR) and monitoring of serological titers may assist the diagnosis.

  • In chronic recurrent nature of the vasculitis, a long-term antiviral therapy may decrease the recurrences and reduce the need of steroids to control the inflammation, although the endpoint of treatment remains unknown.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.

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