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Eric Schneider, Susan G. Elner, Frederik J. Van Kuijk, Mark W. Johnson; Expanding The Spectrum Of Cytomegalovirus-Related Retinopathy: Necrotizing Retinitis Associated With Pan-Retinal Occlusive Vasculopathy In Non-HIV Patients. Invest. Ophthalmol. Vis. Sci. 2011;52(14):2740.
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To characterize a unique cytomegalovirus (CMV)-associated necrotizing retinitis distinguished by pan-retinal occlusive vasculopathy in the setting of limited immune dysfunction.
Retrospective observational case series. CMV was confirmed as the pathogenic agent via polymerase chain reaction analysis of aqueous or vitreous humor samples or via immunohistochemical and electron microscopy analysis of retinal biopsy specimens.
Three non-HIV patients with necrotizing retinitis and severe occlusive vasculopathy were identified. Patient age ranged 42-78 years. Patient histories all suggested a basis for a variable degree of partial immune dysfunction, ranging from advanced age and diabetes mellitus to recent allogeneic stem cell transplant. Localized areas of granular retinal necrosis were noted in all patients as was a variable degree of intraocular inflammation. The level of intraocular inflammation varied inversely with the presumed level of immune dysfunction. An initial clinical diagnosis of acute retinal necrosis (ARN) was made in all patients. In two cases treated empirically for presumed herpes simplex and varicella zoster virus, primary anti-viral therapy failed. Retinitis subsequently regressed in all cases with introduction of ganciclovir therapy following confirmation of CMV in ocular specimens. Two of three patient developed neovascularization due to extensive retinal ischemia.
The clinical expression of CMV-associated retinopathy is strongly related to immune status. In patients with a minimal to intermediate level of immune dysfunction, a mixed clinical picture of intraocular inflammation with pan-retinal occlusive vasculopathy, more characteristic of ARN, and peripheral slowly progressive granular retinitis, more characteristic of classic CMV retinitis is observed. Recognition of this intermediate manifestation of necrotizing viral retinitis should prompt testing for CMV as empiric ARN therapy is unlikely to halt disease progression. Consideration should also be given to prophylactic pan-retinal photocoagulation given the risk of neovascular complications.
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