Optical coherence tomography (OCT): Can be used to localize the vitelliform lesion to the subetinal space, demonstrate thickening of the cone outer segments, and can be used to evaluate for fluid associated with CNV.

2005年2月1日 · Best's vitelliform macular dystrophy is characterized by abnormal retinal deposition of lipofuscin, the exact location of which remains to be definitely elucidated, notwithstanding several clinical and optical coherence tomography (OCT) reports. 1, 2, 3, 4 In this report we present new OCT findings of “pseudohypopyon” and “scrambled-egg ...

2010年12月2日 · Best’s disease is a rare, incurable, autosomal dominant, slowly developing vitelliform (yolk-like) macular dystrophy associated with central visual disturbance and loss. The disease is limited to macular anomalies, excluding systemic associations.

2022年9月7日 · Best disease is an inherited disease that affects the retinas of your eyes. It causes the macula, which is the central part of the retina, to degrade. This means that you can have problems with your central vision, or seeing things that are right in front of you. Best disease may not affect peripheral (side) vision.

2022年1月1日 · OCT Features: OCT can distinguish differences between the various stages of Best disease. In the vitelliform stage, the subretinal material exhibits a mixture of both hyper-reflective and hyporeflective material.

2024年12月16日 · Best disease is characterized by a distinctive bilateral yellow “egg-yolk” lesion in the macula due to the accumulation of lipofuscin. The condition typically presents in childhood or early adulthood and is often associated with a favourable prognosis (2).

Best's vitelliform macular dystrophy is characterized by abnormal retinal deposition of lipofuscin, the exact location of which remains to be definitely elucidated, notwithstanding several clinical and optical coherence tomography (OCT) reports. 1–4 In this report we present new OCT findings of “pseudohypopyon” and “scrambled-egg ...

Conclusions: OCT is a non invasive and useful tool allowing correlations with fundus examination at every stage of the disease. OCT clearly distinguishes the vitelliform stage from the fibrotic stage. In VMD, OCT shows vitelliform material accumulated under the RPE band, these data are in agreement with our knowledge about histopathologic reports.

These new OCT findings could explain the retention of good visual acuity in the "pseudohypopyon stage" and the loss of visual acuity in the "scrambled egg" stage of Best's disease.

From top to bottom are subclinical (a, stage I), vitelliform (b, stage II), pseudohypopyon (c, stage III), vitelliruptive (d, stage IV), and macular atrophy (e, stage V) stages. Beginning in stage...