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Best disease (vitelliform phase)

This picture represents the egg-yolk (vitelliform) phase of this dominantly inherited macular dystrophy. During this phase, the acuity usually remains normal in spite of the significant accumulation of lipofuscin under the macula. (Contributed by Dr. James Walters.)

Associated Disorders

  • Bassen-Kornzweig disease
  • Cushing syndrome
  • Kearns-Sayre syndrome
  • Lawrence-Moon-Biedl syndrome
  • Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS)
  • Ocular paraneoplastic syndromes
  • Refsum disease
  • Usher syndrome
  • gastroesophageal reflux disease
  • posterior vitreous detachment
  • retinal detachment
  • systemic arterial hypertension
  • systemic lupus erythematosus