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Purpose:To report the peripheral abnormalities seen only with indocyanine green angiography in patients with vitelliform macular dystrophy(Best disease,caused by a mutation in the bestrophin gene).Design:Observational case report series.Methods:Eight eyes of four patients,two with only a central macular lesion and two with multifocal lesions,were studied.Results of indocyanine green angiography were compared with findings from ophthalmoscopy and fluorescein angiography.Results:Throughout the fundus periphery,indocyanine green angiography demonstrated a number of hyperfluorescent spots in all eight eyes.The spots were observed in the midperiphery and the periphery in areas with no abnormality visible by ophthalmoscopy or fluorescein angiography.Conclusions:Although Best disease generally causes lesions visible in the posterior pole,the extensive distribution of the hyperfluorescent spots is consistent with the wide-ranging abnormalities of the retinal pigment epithelium,Bruch membrane,and the choroid as seen histopathologically.
Purpose: To report the peripheral abnormalities seen only with indocyanine green angiography in patients with vitelliform macular dystrophy (Best disease, caused by a mutation in the bestrophin gene). Design: To Observational case report series. Methods: Eight eyes of four patients, two with only a central macular lesion and two with multifocal lesions, were studied. Results of indocyanine green angiography were compared with findings from ophthalmoscopy and fluorescein angiography. Results: Throughout the fundus periphery, indocyanine green angiography demonstrated a number of hyperfluorescent spots in all eight eyes. The spots were observed in the midperiphery and the periphery in areas with no abnormality visible by ophthalmoscopy or fluorescein angiography. Conclusions: Although Best disease generally visible lesions in the posterior pole, the extensive distribution of the hyperfluorescent spots is consistent with the wide-ranging abnormalities of the retinal pigment epithelium, Bruch membrane, a nd the choroid as seen histopathologically.