THE PROGRESSIVE OUTER RETINAL NECROSIS SYNDROME - A VARIANT OF NECROTIZING HERPETIC RETINOPATHY IN PATIENTS WITH AIDS

Background: The progressive outer retinal necrosis syndrome is a recen tly recognized variant of necrotizing herpetic retinopathy. This repor t characterizes more fully its clinical features and course. Methods: Using standardized clinical criteria, patients with progressive outer retinal necrosis syndrome from four institutions were identified. Pati ent records were reviewed retrospectively for the following data: medi cal and demographic characteristics, presenting symptoms, physical fin dings, course, responses to treatment, and outcomes. Results: Thirty-e ight patients (65 involved eyes) were studied. All had acquired immune deficiency syndrome. A known history of cutaneous tester was document ed in 22 (67%) of 33 patients. Median CD4 lymphocyte count was 21/mm(3 ) (range, 0-130/ mm(3)). Median follow-up was 12 weeks. The most commo n presenting symptom was unilateral decreased vision (35 of 65 eyes, 5 4%); median visual acuity at presentation was 20/30 (range, 20/20 to n o light perception [NLP]). Anterior chamber and vitreous inflammatory reactions were absent or minimal in all patients. Typical retinal lesi ons were multifocal, deep opacities scattered throughout the periphery , although macular lesions also were present in 21 eyes (32%) at diagn osis. Lesions progressed rapidly to confluence. Initial intravenous an tiviral therapy appeared to reduce disease activity in 17 (53%) of 32 eyes, but treatment did not alter final visual outcome. Visual acuity was NLP in 42 (67%) of 63 eyes within 4 weeks after diagnosis. Retinal detachment occurred in 43 (70%) of 61 eyes, including 13 (93%) of 14 eyes that received prophylactic laser retinopexy. Conclusion: The prog ressive outer retinal necrosis syndrome is characterized by features t hat distinguish it from cytomegalovirus retinopathy, acute retinal nec rosis syndrome, and other necrotizing herpetic retinopathies. Visual p rognosis is poor with current therapies.