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Start free with EleplanStargardt disease
ORPHA:827Disease
Also called Fundus flavimaculatus · Stargardt 1
What it is
A rare ophthalmic disorder that is usually characterized by a progressive loss of central vision associated with irregular macular and perimacular yellow-white fundus flecks, and a so-called ''beaten bronze'' atrophic central macular lesion.
Key facts
- Prevalence
- 1-5 / 10 000 (Europe)
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Inheritance
- Autosomal dominant, Autosomal recessive
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
12- Abnormal choroid morphologyDiagnostic criterion
- Abnormal foveal morphology
- Abnormality of macular pigmentation
- Abnormality of visual evoked potentials
- Central scotoma
- Color vision defect
- Macular degeneration
- Nyctalopia
- Paroxysmal involuntary eye movementsDiagnostic criterion
- Retinal pigment epithelial atrophy
- Retinal pigment epithelial mottling
- Retinal thinning
Common30–79%
3- Aplasia/Hypoplasia of the macula
- Photopsia
- Yellow/white lesions of the maculaDiagnostic criterion
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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