Purpose: To characterize a unique cytomegalovirus (CMV)-associated retinopathy in patients with limited immune dysfunction.
Methods: 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 analysis of retinal biopsy specimens.
Results: Five non-HIV patients with granular necrotizing retinitis, vitritis, and severe occlusive vasculopathy were identified. Patient histories all suggested a basis for limited immune dysfunction including advanced age (n = 4), diabetes mellitus (n = 4), and noncytotoxic immunotherapy (n = 3). Diagnosis of CMV retinitis was delayed in all cases and patients received either no antiviral therapy (n = 2) or incorrect antiviral therapy (n = 3) for presumed herpes simplex/varicella zoster-related acute retinal necrosis. Retinitis subsequently regressed in all cases with introduction of systemic ganciclovir/valganciclovir (n = 5) and/or intravitreal foscarnet (n = 2). Four of five patients developed neovascularization because of extensive retinal ischemia.
Conclusion: The clinical expression of CMV-associated retinopathy is strongly related to immune status. In patients with limited immune dysfunction, a mixed clinical picture of intraocular inflammation with panretinal occlusive vasculopathy, more characteristic of acute retinal necrosis, and peripheral slowly progressive granular retinitis, more characteristic of classic CMV retinitis, is observed. Recognition of this atypical clinical presentation, which the authors term chronic retinal necrosis, should prompt molecular testing for CMV to determine the appropriate antiviral therapy. Consideration should also be given to prophylactic panretinal photocoagulation in such eyes, given the high risk of neovascular complications.
Summary: Five cases of atypical cytomegalovirus (CMV) retinitis—characterized by limited granular retinitis, vitritis, and severe panretinal occlusive vasculopathy—are identified in patients with histories suggestive of partial immune dysfunction. Initial therapy was commonly directed against herpes simplex/varicella zoster for presumed acute retinal necrosis. Such cases of cytomegalovirus chronic retinal necrosis in non-HIV patients are part of a spectrum of cytomegalovirus-associated retinopathy, with the clinical phenotype determined by extant immune function.
*Department of Ophthalmology and Visual Sciences, University of Michigan, W.K. Kellogg Eye Center, Ann Arbor, Michigan;
†Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota;
‡Department of Ophthalmology, Mount Sinai School of Medicine, New York, New York; and
§Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts.
Reprint requests: Mark W. Johnson, MD, Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center—University of Michigan Hospitals, 1000 Wall Street, Ann Arbor, MI 48105; e-mail: email@example.com
Supported by Research to Prevent Blindness Unrestricted Grant (E.V.K.).
None of the authors has any financial interests to disclose.