Classification and management of coats disease: The 2000 Proctor Lecture

PURPOSE: To review the methods and results of management in a large series of patients with Coats disease, to determine risk factors for poor visual o utcome and enucleation, and to propose a practical classification of Coats disease. METHODS: In a retrospective consecutive series in 150 patients, Coats disea se was defined as idiopathic retinal telangiectasia with intraretinal or su bretinal exudation without appreciable signs of retinal or vitreal traction . We reviewed our experience with management, including observation, laser photocoagulation, cryotherapy, and various techniques of retinal detachment surgery and improvement or stability was achieved in 76% of eyes, and fina l visual acuity was 20/50 or better in 11 eyes (14%), 20/60 to 20/100 in ei ght (6%), 20/200 to finger counting in 30 (24%), and hand motion to no ligh t perception in 49 (40%) Enucleation was ultimately necessary in 20 eyes (1 6%). Risk factors predictive of poor visual outcome (20/200 or worse) included p ostequatorial (P =.01), diffuse (P =.01), or superior (P =.04) location of the telangiectasias and exudation, failed resolution of subretinal fluid af ter treatment (P =.02), and presence of retinal macrocysts (P =.02). The ma in risk factors for enucleation were elevated intraocular pressure (greater than 22 mm Hg; P less than or equal to .001) and iris neovascularization ( P less than or equal to .001). Coats disease was classified into stage 1, telangiectasia only; stage 2, te langiectasia and exudation (2A, extrafoveal exudation; 2B, foveal exudation ) stage 3, exudative retinal detachment (3A, subtotal; 3B, total); stage 4, total detachment and secondary glaucoma; and stage 5, advanced end-stage d isease. Poor visual outcome (20/200 or worse) was found in 0% of eyes with stage 1, 53% with stage 2, 74% with stage 3, and 100% of stages 4 and 5 Coa ts disease. Enucleation was ultimately necessary in 0% of stages 1 and 2, 7 % of stage 3, 78% of stage 4, and 0% of stage 5 disease. CONCLUSIONS: Carefully selected treatment can anatomically stabilize or imp rove the eye with Coats disease in 76% of eyes. However, poor visual outcom e of 20/200 or worse commonly results. Patients who present with stages 1 t o 3 Coats disease have the best visual prognosis, and patients with stages 4 and 5 have a poor visual prognosis. (C) 2001 by Elsevier Science Inc. All rights reserved.).