Clinical variation and complication of Coat dieae in 150 cae: the 2000 anford Gifford Memorial Lecture

PURPOE: The purpoe of thi report i to review the clinical variation and natural coure of Coat dieae, uing trict diagnotic guideline. METHOD: In a retropective, conecutive erie, 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 the clinical feature, complication, and diagnotic approache to Coat dieae. REULT: In 150 conecutive patient (158 eye), Coat dieae wa diagnoed at a median age of 5 year (range, 1 month to 63 year), occurred in 114 male (76%), and wa unilateral in 142 patient (95%). There wa no predilection for race or laterality. The mot common referral diagnoe were Coat dieae in 64 cae (41%) and retinoblatoma in 43 (27%). The firt ymptom or ign wa decreaed viual acuity in 68 cae (34%), trabimu in 37 (23%), leukocoria in 31 (20%), and 13 patient (8%) were aymptomatic. Viual acuity at preentation wa 20/200 to no light perception in 121 eye (76%). The anterior egment wa normal in 142 eye (90%). The retinal telangiectaia involved the midperipheral or peripheral fundu in 156 of the 158 eye (99%) and wa retricted to the macular area in two eye (1%); involved mainly the temporal fundu in 66 eye (42%), inferior fundu in 41 eye (26%), and more than one ector in 34 eye (22%). Retinal exudation wa preent in all 12 clock hour in 86 eye (55%) and ix or more clock hour in 115 eye (73%). There wa a total retinal detachment in 74 eye (47%) and neovacular glaucoma in 12 (8%). Retinal macrocyt were preent in 18 eye (11%), a vaoproliferative tumor in nine eye (6%) and retinal neovacularization in four eye (3%). Fluorecein angiography in 49 of the 158 eye (37%) dicloed early hyperfluorecence of the telangiectaia and macular edema in 18 of eye (37%). Ultraonography typically howed a retinal detachment but no olid ma. CONCLUION: Coat dieae i a ditinct clinical entity characterized by idiopathic retinal telangiectaia and retinal exudation. It i uually unilateral, occur motly in young male, and can caue evere viual lo reulting from exudative retinal detachment. The clinician hould follow trict criteria in making the diagnoi, to avoid confuing Coat dieae with other form of exudative retinopathy.