Cytomegalovirus-associated Panretinal Occlusive Vasculopathy without Retinal Necrosis as the Heralding Feature of Good Syndrome.

Purpose: To introduce an atypical occlusive panretinal vasculitis without necrotizing retinitis secondary to cytomegalovirus (CMV) infection as the heralding clinical event for a patient ultimately discovered to have Good Syndrome, a systemic immunodeficiency. Methods: Case report. Results: A 77-year-old male with a history of hypertension, diabetes mellitus, recurrent sinusitis, and oral thrush presented with 3 weeks of headaches and vision loss (20/250) in the right eye. Examination revealed panuveitis, scattered mid-peripheral intraretinal hemorrhages without retinal necrosis, profound retinal vascular nonperfusion, and elevated IOP due to anterior segment neovascularization. Aqueous humor viral PCR analysis revealed a high CMV viral load. Systemic evaluation was negative for HIV, CMV, toxoplasmosis, and syphilis; carotid duplex imaging was normal, leukemia and lymphoma studies were negative; and he was not neutropenic. CD4+ count and immunoglobulin levels were decreased, suggesting a combined B- and T-cell immunodeficiency. Computed tomography of the chest revealed a thymoma, consistent with Good Syndrome, a rare immunocompromising condition. Antiviral therapy did not significantly reverse retinal perfusion. Conclusion: Panretinal occlusive vasculitis without retinal necrosis is a rare phenotype of CMV retinitis. In HIV-negative patients with CMV retinitis, additional immunodeficiency workup is required, and rare combined immunodeficiencies such as Good Syndrome should be included on the differential.