What is a significant ocular manifestation seen in patients with Best's disease?

Best's disease, also known as Best vitelliform macular dystrophy, is primarily characterized by the accumulation of lipofuscin within the retinal pigment epithelium, which leads to certain distinctive ocular manifestations. One of the hallmark features of Best's disease is the presence of subretinal flecks. These flecks are yellowish-white lesions that occur due to the abnormal accumulation of substances beneath the retina.

Patients typically show a yolk-like, vitelliform lesion in the macula during the early stages, but as the condition progresses, subretinal flecks and other changes in the retinal layers can be observed on clinical examination or imaging studies. This accumulation directly relates to the underlying genetic defect affecting the RPE cells and their ability to transport and metabolize lipids.

Understanding the specific ocular manifestations associated with Best's disease is crucial for timely diagnosis and management, as the presence of subretinal flecks serves as a significant marker for this condition. These flecks are more characteristic of Best's disease than other retinal diseases that may lead to varying ocular symptoms but do not specifically present with subretinal flecks.