Título / Title

ADULT FOVEOMACULAR VITELLIFORM DYSTROPHY: SERIES OF TWO CLINICAL CASES REPORT

Introdução / Purpose

Adult Foveomacular Vitelliform Dystrophy is a rare, progressive and bilateral condition characterized by a rounded deposit of sub retinal material that affects patients between the fifth and sixth decades of life. The differential diagnosis is important to exclude some pathologies, such as Age related Macular Degeneration that are most common in this age group and other hereditary conditions that are more common in the first decades of life. Complications such as choroidal neovascularization are uncommon (5 to 10%), but when present worsen vision and visual prognosis. The research with anti-VEGF has brought new perspectives to the treatment of this complication.

Material e Método / Methods

To report two clinical cases and review of the literature.

Resultados / Results

Patient 1: male, 61 years old, with progressive vision loss six months ago. The vision acuity was 20/60 in the right eye and 20/50 in the left eye. The fundoscopy showed a yellowish, rounded and discreetly elevated lesion and the OCT evidenced a RPE elevation with protruding hyperreflexive subfoveal range. Patient 2: male, 50 years old, complained of vision loss in the left eye one year ago. The vision acuity was 20/40 in right eye and 20/70 in the left eye. The fundoscopy showed a rounded lesion in the fovea in the right eye and atrophic region in the macula in the left eye. The Fluorescein Angiography evidenced a window defect in both eyes and the OCT an elevated sub foveal lesion without sub retinal fluid in the right eye.

Discussão e Conclusões / Conclusion

Adult Foveomacular Vitelliform Dystrophy is a rare condition that affects patients in the fifth decade of life and is characterized by the subfoveal viteliform deposit. The diagnosis is important, mainly, to exclude other pathologies. The anti-VEGF comes as an ally in cases of complications such as choroidal neovascularization.

Palavras Chave

Adult Foveomacular Vitelliform Dystrophy; macular degeneration; macular disease