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F. Nesi, M.M. Lai, G.A. Williams; Atypical Presentations of Progressive Outer Retinal Necrosis Masked by Pre–Existing Non–HIV Related Co–Morbidities . Invest. Ophthalmol. Vis. Sci. 2006;47(13):3054.
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To present a case series of progressive outer retinal necrosis (PORN) that masqueraded as pre–existing non–HIV related diseases.
Retrospective review of clinical records and review of literature.
We present two cases of patients with PORN whose diagnoses were confounded by pre–existing diseases. A sixty–eight year– old female with a history of systemic lupus erythematosus presented with decreased vision in both eyes following a course of oral prednisone in addition to her baseline dose of hydroxychloroquine. She was initially diagnosed with retinal vasculitis based on findings of marked arteriole constriction, boxcarring of the veins, perivascular sheathing and intraretinal hemorrhage. She was treated with intravenous dexamethasone and cytoxan. Her visual acuity declined further and she developed features of viral retinitis including retinal whitening and detachment. The vitreous was positive for herpes simplex virus–2 by polymerase chain reaction. PORN was diagnosed and vitrectomy, membrane stripping, silicone oil and gancyclovir placement were performed in her right eye. Despite further intravitreal injections of gancyclovir and foscarnet, the vision declined to no light perception (NLP) in both eyes. The second patient was a forty –nine year –old male with a history of psoriasis treated with etanercept who presented with decreased vision in the right eye. He was initially treated with intravitreal triamcinolone for possible reactivation of a previous toxoplasmosis scar. He subsequently developed fundus findings and fluorescein angiographic features consistent with viral retinits. His visual acuity declined to NLP despite discontinuation of the etanercept and treatment with valacyclovir. A review of the literature discloses that varicella zoster virus represents the etiology in the majority of the reported cases of PORN, particularly in the HIV negative subset. The use of steroids in these patients led to exacerbation of symptoms and poor visual outcomes.
Atypical presentations of PORN can be seen in HIV–negative patients with compromised immune systems. The use of systemic or intravitreal steroids leads to rapid progression of viral retinitis. Therefore, the use of steroids in the treatment of atypical retinal vascular diseases in immunocompromised patients should be closely monitored.
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