Title
UNILATERAL VITELLIFORM MACULAR INJURY
Introduction
Vitelliform Macular Dystrophy, is a rare, bilateral disease of autosomal dominant inheritance. Individuals affected develop a yellow "egg yolk" macular lesion usually in childhood, which eventually breaks down, leaving a geographical atrophic appearance. At the end of the disease, atrophy may be difficult to distinguish from other types of degeneration or macular dystrophies. The disease can be identified at various clinical stages: a pre-vitelliform stage, when there are no substantial eye changes, to choroidal vascularization, which is associated with a poor prognosis. Most patients maintain good visual acuity (VA) throughout the disease.
Methods
A case report with medical record review.
Results
89-year-old white female came complaining of nonspecific eye discomfort. Pathological history of esophageal cancer, with chemotherapy completed months ago and facectomy with IOL implantation in both eyes (BE). Best corrected VA 20/25 BE. Anterior segment presented blepharitis and pseudophakia, topical IOL. The fundus examination showed a yellowish-white lesion in the macular region in the right eye. In the left eye, there were no significant findings. Retinal multimodal evaluation was performed in BE, without significant findings in the OE. On autofluorescence examination, in RE the lesion was short-wave hyperfluorescent. RE autofluorescence the lesion was short-wave hyperfluorescent. Macula OCT demonstrated subretinal median reflectivity content causing cupuliform elevation of the nasal parafoveal neurosensory retina, with signs of degeneration of the outer retinal layers overlying the lesion, and fusiform alteration at the level of the RPE causing posterior optical shading. En-face OCT, at the level of the external limiting membrane presents a lesion with an internal content of medium reflectivity and high reflectivity contours.
Discussion
Unilateral best's disease in elderly patient is a rare condition. Differential diagnosis should be made with other retinal dystrophies and maculopathies.
Keywords
Best's Disease, Retinal dystrophies, Maculopathies
Area
CLINICAL CASE
Authors
LANA MARTINS MENEZES, RAFAEL CICCONI ARANTES, JULIANA ABREU RIO, RICARDO LUZ LEITÃO GUERRA