Cystoid Macular Edema: An Atypical Complication of Adult-Onset Foveomacular Vitelliform Dystrophy

Introduction. Adult-Onset Foveomacular Vitelliform Dystrophy (AOVD) is a bilateral, macular dystrophy. AOVD presents as a subretinal, oval or round, yellowish lesion in macula that manifests between 30 and 50 years of age. It is autosomal dominant with a slowly, progressive decrease in vision. AOVD is a pattern dystrophy of retinal pigment epithelium. AOVD presents as a slight decrease in vision or metamorphopsia later in life and can progress to geographic atrophy of RPE or CNVM. AOVD is associated with several complications and we report the first case of AOVD with subsequent Cystoid Macular Edema. Case Presentation. A 52-year-old Caucasian male presents with intermittent blurry vision and metamorphopsia of both eyes of several years duration. His best corrected acuity was OD 20/20 - OS 20/25. Fundus examination revealed Optic nerve heads that were flat and sharp bilaterally. Round, yellowish subretinal lesion in macula bilaterally with associated irregularity and thickening in macula bilaterally. Deviation from expected. This is the first reported case of Cystoid Macular Edema as a complicaton Adult Onset Foveomacular Vitteliform Dystrophy. Discussion. AOVD presents with a yellowish round sub-foveal lesion made up of lipofuscin being secreted by the retina. Lipofuscin is normal metabolic byproduct of retina, phagocytized by retina. Increased metabolic activity secondary to age, genetics, environment, etc. leads to increasing levels of circulating lipofuscin. Excess lipofuscin stored at level of RPE and presents as a vitelliform lesion. The lesions eventually reabsorb into retinal space and result in atrophy of the RPE Subfoveal choroidal neovascularization. Complications include: vascularized pigment epithelial detachment, Retinal folds, Central Serous Chorioretinopathy, Macular hole/RD, and Cystoid Macular Edema. CME is described as a thickening of macula due to intraretinal edema. Perifoveal capillaries leak abnormally resulting in growth of extracellular space and fluid accumulation in tissue. This causes 29 disruption of inner or outer blood-retinal barrier due to subsequent incompetence of retinal microvasculature. CME is a rarely reported complication of AOVD. Conclusion. AOVD is usually a benign pattern dystrophy of the retina. There are rarely reported complications associated with the condition. Ancillary testing such as the OCT and ERG can be used to aid in the diagnosis of AOVD and CME. Clinicians need to be knowledgeable about these potentially visually significant findings.