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Focal retinal pigment epithelial dysplasia associated with fundus flavimaculatus
Background:
One or more focal dysplastic lesions of the retinal pigment epithelium (RPE) occurred in 15 eyes of 10 patients with fundus flavimaculatus.
Methods:
Review of patient records including an attempt to obtain follow-up information concerning a history of previous ocular trauma.
Results:
Mild antecedent ocular trauma occurred to the eye with a dysplastic lesion in two patients. Dysplastic lesions were most frequently solitary and located temporal to the macula. Subretinal neovascularization accompanied two of the dysplastic lesions. The lesions were multifocal and present bilaterally in two patients.
Conclusions:
In fundus flavimaculatus, progressive lipofuscin storage is responsible for engorgement and hypertrophy of the RPE. Dysplastic lesions of the RPE probably result from reactive hyperplasia and fibrous metaplasia of RPE cells in response to acute disruption of fragile, hypertrophied RPE cells that may be enormously enlarged in the area of yellow flecks. This disruption may occur in response to trauma, focal inflammation, or other localized stimuli. Patients with fundus flavimaculatus should be cautioned concerning the possible role of trauma in causing dysplastic changes in the RPE and visual loss.