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Programação » Relatos de Caso (Poster)

Código: RC 172
Área Técnica: Retina

INSTITUIÇÃO ONDE FOI REALIZADO O TRABALHO:

AUTOR PRINCIPAL:

CO-AUTOR(ES):

TÍTULO:
IMAGING AND PHYSIOLOGICAL STUDY IN BENIGN CONCENTRIC ANNULAR MACULAR DYSTROPHY: A CASE REPORT

OBJETIVO:
To describe multifocal electroretinogram (mfERG), visual field test, spectral domain-optical coherence tomography (SD-OCT) and fundus findings in a patient with benign concentric annular macular dystrophy (BCAMD).

RELADO DE CASO:
EPS, female, 35 years old, was referred for bilateral visual impairment for 10 years. There was no history of systemic or family eye diseases. Corrected visual acuity was 20/25 and ocular pressure was 12 mmHg in both eyes. There was no alterations in anterior segment. Fundoscopic examination showed foveal annular aspect. In fluorescein angiography (FA), we observed an perifoveal area of hyperfluorescence corresponding to the area of hypopigmentation of the retinal pigment epithelium (RPE). Perifoveal dotted areas of hyperfluorescence with diffuse hypofluorescence were found. Those dottet areas were due to the late impregnation and they did not show clinical visible lesions. That pattern of uptake is indicative of a generalized choriocapillary and RPE dystrophies. Full-field ERG was normal, but mfERG showed decreased electrical activity in the central retina, corresponding to the region of reduced sensitivity in visual field test. The autofluorescence showed a defect in the RPE more extensive than seen in the FA, demonstrating that this is probably the origin of the lesion, that secondarily involve overlying photoreceptors. The patient's SD-OCT showed a discontinuity line in outer and inner segments of photoreceptors in both eyes. The macular thickness map showed preservation of foveal thickness and thinning of the perifoveal retina.

CONCLUSÕES:
BCAMD is a very rare autosomal dominant disease with a high clinical variability and well marked findings. The disease seems to affect more extensively the RPE and lead to the suffering of the macular photoreceptors. Those are very important aspects to establish the correct diagnosis.

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