Título

MULTIFOCAL PATTERN DYSTROPHY SIMULATING FUNDUS FLAVIMACULATUS: CASE EVOLUTION REPORT

Introdução / Purpose

To illustrate the evolution of a multifocal pattern of macular dystrophy simulating fundus flavimaculatus

Material e Método / Methods

observation

Resultados / Results

The indirect ophthalmoscopy examination and fundus evaluation, sub-retinal deposits were observed, with yellowish coloration and with a relative radiated pattern, besides these recurrent ones affecting the equatorial region with flecks around vascular arcades. The fovea of RE has areas of chorioretinal atrophy and LE the fovea was spored (figura 1).

Infrared reflectance imaging showed hypoautofluorescence corresponding to the atrophic area. Fundus autofluorescence imaging showed hypoautoflourescence corresponding to the atrophic areas and hyperautofluorescence corresponding to flecks. Fundus fluorescence angiography at late phase showed diffuse transmission defects and staining of the atrophic area. There was no choroidal silence.

On Optic coherence tomography, some of the Stargardt-like flecks appear as a highly reflective focal thickening of the hyper-reflective outer red line, with diffuse alteration of the choriocapillar EPR complex with several protrusion points sub EPR.

Discussão e Conclusões / Discussion

While genotypically similar to other pattern dystrophies, multifocal pattern of macular dystrophy simulating fundus flavimaculatus is phenotypically unique with a close resemblance, funduscopically, to fundus flavimaculatus and Stargardt’s disease. The test of choice to aid in diagnosis is fluorescein angiography, which shows normal choroidal flush in multifocal pattern dystrophy and the pathognomonic dark choroid in fundus flavimaculatus and Stargardt’s disease. This delineation is key because of the better visual prognosis associated with multifocal pattern dystrophy.
Management of the dystrophy involves patient education, genetic counseling, referral to a retina specialist and treatment of a choroidal neovascular membrane, in the rare instance that it occurs. In the event of impaired vision, low vision rehabilitation to aid in activities of daily living its also a important

Palavras Chave

PATTERN DYSTROPHY, SIMULATING FUNDUS FLAVIMACULATUS, MULTIFOCAL PATTERN DYSTROPHY, DISTROFIA MACULAR MULTIFOCAL.

Area

CLINICAL RETINA