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The most common form of inherited juvenile macular degeneration. Characterized by a reduction of central vision with a preservation of peripheral vision. Onset before the age of 20 years; macula presents yellow-white spots of irregular shapes.

Stargardt Disease; Stargardt Macular Degeneration; Familial Juvenile Macular Degeneration; Juvenile Hereditary Disciform Macular Degeneration; Fundus Flavimaculatus; Central Retinitis Pigmentosa.

This autosomal recessive transmitted disease with gene map locus 1p21-p13 is the most common cause of juvenile-onset macular degeneration and affects at least 1 in 20,000 children older than the age of 6 years. The disorder is characterized by subretinal deposition of lipofuscin-like material (termed “flecks”) around the macular area. There is evidence that the disease is caused by a mutation in the retina-specific ATP-binding cassette transporter (ABCR) gene.

In general, the age at manifestation of the disease is between 6 and 20 years with decreased central vision being the first symptom, although some patients do not suffer from any symptoms (loss of color vision, loss of peripheral vision, photophobia, paracentral scotoma, night blindness) until the age of 40 years. Fundus flavimaculatus is the descriptive term for this macular degeneration surrounded by white-yellowish flecks and spots distributed all over the fundus, although some researchers consider fundus flavimaculatus to be an allelic variant of Stargardt disease with differences in age at onset (between 17 and 60 years), severity, and clinical course (more progressive). The rapidly progressive disease occurs usually bilaterally and symmetrically and results in legal blindness in 50% of patients by the age of 50 years. No effective treatment is available.

Especially in younger patients undergoing ophthalmic examination under general anesthesia, oculocardiac reflex with profound bradycardia should be expected. No other specific anesthetic considerations are expected to arise from this disease.

Armstrong J, Meyer D, Xu S, et al: Long-term follow-up of Stargardt's disease and fundus flavimaculatus. Ophthalmology 105:448, 1998.  [PubMed: 9499775]
Hadden OB, Gass JD: Fundus flavimaculatus and Stargardt's disease. Am J Ophthal 82:527, 1976.  [PubMed: 1086060]
Kaplan J, Gerber S, Larget-Piet D, et al: A gene for Stargardt's disease (fundus flavimaculatus) maps to the short arm of chromosome 1. Nat Genet 5:308, 1993.  [PubMed: 8275096]

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