Title
VITELLIFORM MACULAR DYSTROPHY - CASE REPORT
Introduction
Vitelliform macular dystrophy is a rare bilateral pathology with autosomal dominant mode of transmission. It has variable expression and penetrance, which affects the retinal pigmented epithelium (RPE). In electrophysiological studies, it shows changes in the electroculogram. Among the atypical forms of the disease, those with asymptomatic presentation, whose diagnosis is made through electrophysiology and those with multiple lesions should be considered.
Methods
E.E.L, 49 years old, male, with a complaint of progressive decrease in visual acuity in both eyes (AO) in 6 months. No comorbidities. Better visual acuity (VA) of 20/200 in the right eye and 20/100 in the left eye. Fundoscopy showed multiple yellowish-white, rounded, subretinal lesions in the posterior pole and in the vascular arches with a finding of subretinal fluid (FSR) in a greater lesion in the AO foveal area. An optical coherence tomography (OCT) registered subretinal fluid in AO. Complete blood exam count was also requested to search for immunodeficiencies, which came up without abnormalities. Syphilis, tuberculosis, toxoplasmosis, cytomegalovirus and rapid HIV test were carried out.
Results
The ophthalmological findings despite the non-typical lesions of Best's vitelliform macular dystrophy, this was introduced in the diagnostic hypotheses of the research arsenal. Therefore, the patient underwent an electroculogram (EOG) that presented a borderline pattern Best's disease or Best’s vitelliform macular dystrophy is the second most common macular dystrophy, after Stargardt's disease. Onset may occur in childhood or decades later.
Discussion
Currently, there is no treatment for this condition. The prognosis is generally good until middle age, from which point visual acuity declines in one or both eyes due to choroidal neovascularization, scarring or geographic atrophy.
Keywords
dystrophy macular Vitelliform
Area
CLINICAL CASE
Authors
ADRIANO CABRAL DE VASCONCELOS, FABIANA KARLA AQUINO DA COSTA, ANA PAULA LIRA E CESAR DA CUNHA, EDSON HIDEKI NAKAHARA, BRUNO PACHU RAIA DOS SANTOS, VIVIANE GUEDES