Longitudinal characterization and treatment response of retinal arterial macroaneurysms in adult-onset coats disease

PURPOSE: To perform longitudinal analysis of retinal arterial macroaneurysms in 3 patients with adult-onset Coats disease. OBSERVATIONS: Three eyes of three patients with adult-onset Coats disease were followed longitudinally for 4–15 years. Ultra-widefield images and montage color fundus photographs of affected eyes were analyzed. Size, retinal location, and grading for predominant characteristic (hemorrhagic, exudative, or quiescent) of each individual macroaneurysm were followed longitudinally from the time of onset. Fifty-one individual retinal arterial macroaneurysms were identified. The distance of any lesion-associated hemorrhage or exudation present from the foveal center was measured. Macroaneurysms were located in all quadrants of the retina, with the majority (37/51) graded as hemorrhagic at lesion onset. Hemorrhagic and exudative macroaneurysms that entered the quiescent phase remained quiescent for an average of 26 months. Seven macroaneurysms were found to have hemorrhage or exudation that came within 125 μm of the fovea and all three eyes followed demonstrated a longitudinal decrease in visual acuity despite laser and intravitreal injection therapy. At the initial visit, visual acuities ranged from 20/40 to 20/200, but decreased to 20/80 to 20/320 by the last follow-up visit. CONCLUSION AND IMPORTANCE: There are many challenges in treating patients with adult-onset Coats disease. Long-term loss of visual acuity often results from sequelae of hemorrhage and exudation affecting the macula.