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RETINAL DYSTROPHIES AND SYNDROMES

Usher Syndrome

Clinical Case 02

Usher Syndrome - Clinical Case 02

Fundus photograph of a 32-year-old patient with deafness and poor vision - Usher Syndrome type I. Note the pale disc, extensive areas of RPE atrophy, whitish deposits in the macula periphery and thin vessels.

Usher Syndrome - Clinical Case 02

Autofluorescence image showing a pattern of macular hyperfluorescence, in the same case.

Evaluations

1st evaluation

20 0

09 04 2010 - Anterior Segment Photo

Anterior segment photograph of a 32-year-old patient with deafness and poor vision - Usher Syndrome type I.

Anterior segment photograph of a 32-year-old patient with deafness and poor vision - Usher Syndrome type I.

09 04 2010 - Autofluorescence

Autofluorescence image showing a pattern of macular hyperfluorescence which contrasts with a speckled pattern of predominant hyperautofluorescence in the periphery. These hypoautofluorescent areas tend to become coalescent.

Autofluorescence image showing a pattern of ring macular hyperfluorescence.

Autofluorescence image showing a pattern of macular hyperfluorescence which contrasts with a speckled pattern of predominant hyperautofluorescence in the periphery. These hypoautofluorescent areas tend to become coalescent.

Autofluorescence image showing a pattern of macular hyperfluorescence, in the same case.

09 04 2010 - Colour Retinography

Ultra-widefield photograph showing large areas of RPE atrophy at the periphery and some pigmented spicules in the mid-peripehry

Ultra-widefield photograph showing large areas of RPE atrophy at the periphery and some pigmented spicules in the mid-peripehry

Fundus photograph. Note the pale disc, extensive areas of RPE atrophy, whitish deposits in the macula periphery and thin vessels.

Note the pale disc, extensive areas of RPE atrophy, whitish deposits in the macula periphery and thin vessels.

09 04 2010 - Infrared

09 04 2010 - Microperimetry

09 04 2010 - OCT B-Scan (Longitudinal)

OCT line-scan showing decrease in reflectivity and disorganization of the retinal outer layers, affecting predominantly the photoreceptor cells and the adjacent RPE. There is a relative structural preservation of the retinal inner layers.

OCT line-scan showing decrease in reflectivity and disorganization of the retinal outer layers, affecting predominantly the photoreceptor cells and the adjacent RPE.

09 04 2010 - Red-free

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