Posterior Segment
Stargardt Disease
Also known as: stargardt disease, stargardt macular dystrophy, ABCA4 retinopathy, ABCA4-associated retinopathy, fundus flavimaculatus, juvenile macular dystrophy, retinal flecks
Filed as: Soon
Source-cited from the record's own evidence list. Clinical content programme led by Dr Ankit Mathur, PhD, Grad Cert Ocu Thera, B.S. Optom. How the clinical content is governed
Clinical decision support only
Overview
Stargardt disease (ABCA4-associated retinopathy) is an inherited macular dystrophy, mostly autosomal recessive, that most often starts in childhood although adult-onset forms occur. Yellow-white retinal flecks around the macula accompany macular atrophy, and central vision falls slowly in both eyes. The optometry role is to recognise the pattern, image it, and refer so that the diagnosis can be established and the family counselled.
Recognition
Symptoms: what patients report
- Slow, painless loss of central vision in both eyes, often first noticed at school age.
- Acuity reduced out of proportion to an early, subtle fundus appearance.
- Adult-onset cases present later and are usually milder.
Signs: what the examination shows
- Yellow-white flecks around the macula, with a variable spread into the mid-periphery.
- Macular atrophy, relatively symmetrical between the eyes.
- OCT: flecks appear as focal thickening at the RPE; later the outer retinal layers thin. The subfoveal photoreceptor layer may be attenuated or disrupted.
- Fundus autofluorescence: reduced signal centrally with bright (hyper-autofluorescent) flecks.
What OptoGuide™ covers for stargardt disease
- Pattern reasoning: what this combination of findings points to, and its differentiators
- Don't-miss risks and escalation triggers
- Management tiers with linked Australian therapeutics
- Referral urgency, specialty, and letter drafting