Claification and management of Coat dieae: the 2000 Proctor Lecture

PURPOE: To review the method and reult of management in a large erie of patient with Coat dieae, to determine rik factor for poor viual outcome and enucleation, and to propoe a practical claification of Coat dieae. METHOD: In a retropective conecutive erie in 150 patient, Coat dieae wa defined a idiopathic retinal telangiectaia with intraretinal or ubretinal exudation without appreciable ign of retinal or vitreal traction. We reviewed our experience with management, including obervation, laer photocoagulation, cryotherapy, and variou technique of retinal detachment urgery and enucleation. The anatomic outcome, complication of treatment, viual reult, and reaon for enucleation were tabulated. Factor predictive of poor viual outcome (20/200 or wore) and enucleation were determined uing Cox proportional hazard regreion model. Baed on thee obervation, a taging claification of Coat dieae, applicable to treatment election and ocular prognoi, i propoed. REULT: In 117 patient (124 eye) with a mean follow up of 55 month (range, 6 month to 25 year) primary management wa obervation in 22 eye (18%), cryotherapy in 52 (42%), laer photocoagulation in 16 (13%), variou method of retinal detachment urgery in 20 (17%), and enucleation in 14 (11%). Anatomic improvement or tability wa achieved in 76% of eye, and final viual acuity wa 20/50 or better in 17 eye (14%), 20/60 to 20/100 in eight (6%), 20/200 to finger counting in 30 (24%), and hand motion to no light perception in 49 (40%) Enucleation wa ultimately neceary in 20 eye (16%). Rik factor predictive of poor viual outcome (20/200 or wore) included potequatorial (P = .01), diffue (P = .01), or uperior (P = .04) location of the telangiectaia and exudation, failed reolution of ubretinal fluid after treatment (P = .02), and preence of retinal macrocyt (P = .02). The main rik factor for enucleation were elevated intraocular preure (greater than 22 mm Hg; P le than or equal to .001) and iri neovacularization (P le than or equal to .001). Coat dieae wa claified into tage 1, telangiectaia only; tage 2, telangiectaia and exudation (2A, extrafoveal exudation; 2B, foveal exudation) tage 3, exudative retinal detachment (3A, ubtotal; 3B, total); tage 4, total detachment and econdary glaucoma; and tage 5, advanced end-tage dieae. Poor viual outcome (20/200 or wore) wa found in 0% of eye with tage 1, 53% with tage 2, 74% with tage 3, and 100% of tage 4 and 5 Coat dieae. Enucleation wa ultimately neceary in 0% of tage 1 and 2, 7% of tage 3, 78% of tage 4, and 0% of tage 5 dieae. CONCLUION: Carefully elected treatment can anatomically tabilize or improve the eye with Coat dieae in 76% of eye. However, poor viual outcome of 20/200 or wore commonly reult. Patient who preent with tage 1 to 3 Coat dieae have the bet viual prognoi, and patient with tage 4 and 5 have a poor viual prognoi