Visual loss in cytomegalovirus retinitis caused by cystoid macular edema in patients without the acquired immune deficiency syndrome

Purpose: Although serous macular exudation has been described in patients with the acquired immune deficiency syndrome (AIDS) with active cytomegalovirus (CMV) retinitis, cystoid macular edema (CME) is not encountered in this clinical setting. In contrast to these findings, we describe vision loss due to CME occurring in immunosuppressed patients without AIDS treated for CMV retinitis. Methods: Three patients (four eyes) with systemic immunodeficiency presenting with vision loss underwent ophthalmologic examination, including fundus photography and fluorescein angiography. Systemic evaluation was performed to establish the etiology of immunodeficiency and to rule out human immunodeficiency virus infection. Patients were treated with topical corticosteroid and nonsteroidal anti-inflammatory medications for CME. Results: All patients had severe generalized immune deficiency, related either to drug-induced immunosuppression or primary immunodeficiency. Laboratory studies confirmed the presence of systemic CMV infection. Affected eyes had mild reduction of central vision (range, 20/40 to 20/60). Three of four affected eyes had resolving CMV retinitis outside the posterior pole with mild panuveitis. These eyes showed CME on clinical examination and fluorescein angiography. The CMV lesions regressed after reduction of immunosuppressive agents or after systemic antiviral treatment. Response of CME to topical anti-inflammatory medication was variable. Conclusions: Cystoid macular edema can occur in the setting of resolving CMV retinitis in patients with immunodeficiency other than AIDS. This entity is distinct from serous macular exudation, which can occur in patients with AIDS with active CMV retinitis involving the posterior pole. The disparity between patients with and without AIDS in the development of CME may be important in understanding the pathogenesis of CME.