Retina and optic nerve

Stargardt disease

3:2312 visual cues3 clinical photosFree lesson

In this image

Each number marks an object in the image and the finding it stands for.

Yellow starStargardt disease0:00
StudentJuvenile macular dystrophy0:04
DartboardBull’s-eye maculopathy0:15
GoldfishYellow pisciform flecks0:25
Overflowing trashLipofuscin accumulation0:44
Peanut butter cupUsually autosomal recessive0:55
BlocksABCA41:03
BlackboardDark choroid on angiography1:14
CameraFundus autofluorescence1:37
Clean clock ringPeripapillary sparing1:59
Bronze medalBeaten-bronze appearance2:09
Vitamin AVitamin A supplementation2:15
ReviewEvery marker, in order2:26

Transcript

The narration, grouped by cue. Select a sentence to play from there.

Yellow star0:00
That yellow star on the assignment stands for Stargardt disease.
Student0:04
The student is young because we're dealing with the most common juvenile macular dystrophy. Central vision typically worsens progressively, starting relatively early in life.
Dartboard0:15
Across the room, the dartboard brings us to the macula. Central macular and RPE atrophy may produce a bull's-eye pattern, known as bull's-eye maculopathy.
Goldfish0:25
The goldfish in the tank bring in the flecks, which are yellow and shaped like fish. Compare their outline with the flecks in this color fundus photograph. Pisciform means fish-shaped, and these characteristic yellow flecks lie at the level of the RPE. The goldfish help you remember both their color and their shape.
Overflowing trash0:44
The overflowing trash can shows lipofuscin piling up inside the RPE cells. That abnormal buildup leads to progressive damage to the RPE and photoreceptors.
Peanut butter cup0:55
For inheritance, there's a Reese's Peanut Butter Cup in the classroom. Classic Stargardt disease is usually autosomal recessive.
Blocks1:03
Beside it, read the four alphabet blocks as A, B, C, A, four. ABCA4 is responsible for most classic autosomal recessive Stargardt disease.
Blackboard1:14
The dark blackboard returns us to the lipofuscin buildup. This time, we're looking at its effect on fluorescein angiography. In these angiograms, the dark choroid is the feature to compare with the blackboard. Lipofuscin-laden RPE blocks the underlying choroidal fluorescence, producing that dark appearance. So the board's darkness helps you remember the blocking effect.
Camera1:37
The camera brings in fundus autofluorescence, another way to look at the lipofuscin abnormalities. See how the camera photographs brightly glowing goldfish in the drawing. Beside it is a fundus autofluorescence image. This imaging method highlights the characteristic lipofuscin abnormalities, making it particularly useful for evaluating Stargardt disease.
Clean clock ring1:59
Two more details round out the imaging clues. The clean ring around the classroom clock helps us remember peripapillary sparing, a characteristic imaging feature.
Bronze medal2:09
And the battered bronze medal gives central macular atrophy its classic beaten-bronze appearance.
Vitamin A2:15
Finally, notice the teacher pushing away the vitamin A bottle. In ABCA4-associated Stargardt disease, avoid excessive vitamin A supplementation.
Review2:26
Now let's review what we've learned. In Stargardt disease, central vision typically declines progressively, relatively early in life. Bull's-eye maculopathy may develop from central macular and RPE atrophy, with yellow pisciform flecks at the RPE. Abnormal lipofuscin accumulation progressively damages the RPE and photoreceptors. Classic disease is usually autosomal recessive, and ABCA4 accounts for most classic autosomal recessive cases. On fluorescein angiography, lipofuscin-laden RPE blocks the choroidal fluorescence and gives a dark choroid, and fundus autofluorescence highlights the lipofuscin abnormalities. Add characteristic peripapillary sparing, and the classic beaten-bronze appearance of central macular atrophy. Finally, avoid excessive vitamin A supplementation in ABCA4-associated disease.

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Clinical image credits

Openly licensed photographs used in this lesson, with what was changed.

Saket Y. Maheshwari; Swarupa Chakole. A Rare Occurrence of Stargardt Disease in a Quadragenarian Adult. Cureus 2022. Figure 3a.
CC BY 4.0 Article DOI
Rectangular panel extraction only; no image retouching, recoloring, sharpening, generative processing, or clinical markers added. Original figure annotation and panel letters retained.
José J. López-Fontanet; Gabriel Guardiola Dávila; Natalio Izquierdo; Armando Oliver. Strabismus in an Adolescent With Stargardt Disease: An Atypical Presentation. Cureus 2024. Figure 3, panels A and B.
CC BY 4.0 Article DOI
Full original A/B image retained; decoded to PNG without pixel edits.
Saket Y. Maheshwari; Swarupa Chakole. A Rare Occurrence of Stargardt Disease in a Quadragenarian Adult. Cureus 2022. Figure 3b.
CC BY 4.0 Article DOI
Rectangular panel extraction only; no image retouching, recoloring, sharpening, generative processing, or clinical markers added. Original figure annotation and panel letters retained.