Butterfly-Shaped Pigment Dystrophy

Outline
Case Example
Differential Diagnosis

Outline

Butterfly-shaped pigment dystrophy is characterised by the presence of a series of linear yellow deposits that resemble the appearance of butterfly wings.

Vision is usually relatively unaffected until after the seventh decade of life when photoreceptor and RPE atrophy develops at the macula.

Case Example

Case 1

A 50-year-old Asian male with visual acuity of 6/4.8- (20/15-) in each eye.

Fundus photograph and red-free image (right eye)

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Fundus photograph and red-free image (right eye)

Fundus photography shows mixed, hypo and hyper pigmentary changes at the macula.

Fundus photograph and red-free image (left eye)

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Fundus photograph and red-free image (left eye)

Fundus photography shows mixed, hypo and hyper pigmentary changes at the macula.

Optomap and fundus autofluorescence image

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Optomap and fundus autofluorescence image

FAF shows linear lesions of hyperautofluorescence at the macula resembling the wings of a butterfly. The hypoautofluorescent areas coincide with RPE atrophy.

Optomap and fundus autofluorescence image

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Optomap and fundus autofluorescence image

FAF shows linear lesions of hyperautofluorescence at the macula resembling the wings of a butterfly. The hypoautofluorescent areas coincide with RPE atrophy.

Spectralis OCT volume and line scan (right macula)

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Spectralis OCT volume and line scan (right macula)

OCT shows small RPE elevations corresponding to the hyper-FAF linear lesions, and disruption of the inner segment ellipsoid zone and outer retinal atrophy in the central macula.

Spectralis OCT volume and line scan (left macula)

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Spectralis OCT volume and line scan (left macula)

OCT shows small RPE elevations corresponding to the hyper-FAF linear lesions, and disruption of the inner segment ellipsoid zone and outer retinal atrophy in the central macula.

Differential Diagnosis

Butterfly-Shaped Pigment Dystrophy

Outline

Case Example

Fundus photograph and red-free image (right eye)

Fundus photograph and red-free image (left eye)

Optomap and fundus autofluorescence image

Optomap and fundus autofluorescence image

Spectralis OCT volume and line scan (right macula)

Spectralis OCT volume and line scan (left macula)

Differential Diagnosis

Best disease (vitelliruptive stage)

Stargardt Disease and Fundus Flavimaculatus

Multifocal pattern dystrophy simulating Stargardt disease

Late (neovascular) AMD

Late AMD (Geographic Atrophy)

Chronic central serous chorioretinopathy (CSCR)

Pachychoroid pigment epitheliopathy (PPE)

Desferrioxamine retinopathy

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