Posterior Segment
Central Areolar Choroidal Dystrophy
Also known as: central areolar choroidal dystrophy, CACD, central areolar atrophy, hereditary macular atrophy, macular atrophy without drusen
Filed as: Routine
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
Central areolar choroidal dystrophy is an autosomal dominant macular dystrophy presenting in late childhood or early adulthood. Fine pigment change beside the fovea slowly becomes a sharply outlined patch of atrophy of the RPE and choriocapillaris in each eye, with no flecks and no drusen. Vision falls in both eyes in the fourth and fifth decades. The optometry role is to tell it from geographic atrophy of age-related macular degeneration, image it, refer to establish the diagnosis, and support function.
Recognition
Symptoms: what patients report
- Slow bilateral central blur and reading difficulty, usually from the thirties or forties.
- Early disease may be symptom-free.
- Central scotoma in later stages.
Signs: what the examination shows
- Early: subtle pigment mottling next to the fovea in both eyes.
- Later: a round or oval, well-defined area of atrophy involving the RPE and choriocapillaris.
- No flecks and no drusen.
- OCT: partial loss of the outer nuclear layer with atrophy of the photoreceptor layer and of the RPE and Bruch membrane complex.
- Fundus autofluorescence: speckled signal, with sharply bordered dark areas where atrophy is established.
What OptoGuide™ covers for central areolar choroidal dystrophy
- 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