Best disease (best macular degeneration; vitelliruptive macular dystrophy; polymorphic macular degeneration of braley; vitelliform dystrophy) 141

General: Up to 7 years of age; a type of heredomacular dystrophy; autosomal dominant with variable expressivity.

Ocular: Egg yolk lesion at macula, later absorbed to leave atrophic scar; hemorrhagic or serous exudates beneath pigment epithelium; hyperopia; esotropia; strabismic amblyopia; unusual associations with full-thickness macular hole and extramacular multifocal vitelliform disease have been reported.

Best F. Uber eine Hereditase Maculaffektion; Beitrage zur Vererbungslehie. Z Augenheilkd 1905; 13: 199.

Galianos SO, et al. Multifocal Best's disease and sickle cell trait. Ann Ophthalmol 1981; 13:1181-l183.

Glacet-Bernard A, Coscas G. Full-thickness macular hole and retinal detachment complicating Best's disease. Eur J Ophthalmol 1993; 3:53-54.

Kingham JO, Lochen GP. Vitelliform macular degeneration. Am J Ophthalmol

Merin S. Best's vitelliform macular dystrophy. In: Merin S, ed. Inherited Eye Diseases: Diagnosis and Clinical management. New York: Marcel Dekker, 1991:151-l59.

Miller SA, et al. Choroidal neovascular membrane in Best's vitelliform macular dystrophy. Am J Ophthalmol 1976; 82:252.

Miller SA. Multifocal Best's vitelliform dystrophy. Arch Ophthalmol 1977; 95:984-990.

Mohler CW, Fine SL. Long-term evaluation of patients with Best's vitelliform dystrophy. Ophthalmology 1981; 88: 692-699.

Pece A, et al. Best's multiple vitelliform degeneration. Int Ophthalmol 1992; 16:459-464.

Seddon JM, Sharma S, Chong S, et al. Phenotype and genotype correlations in two Best families. Ophthalmology 2003; 110: 1724-l731.