Best disease presenting as subretinal pigment epithelium hyperreflectivite lesion on spectral-domain optical coherence tomography: Multimodal imaging features

Eur J Ophthalmol. 2022 Sep;32(5):2702-2711. doi: 10.1177/11206721211055961. Epub 2021 Nov 20.


Purpose: To report clinical and multimodal imaging features of Best disease in patients presenting with subretinal pigment epithelium hyperreflective lesions.

Design: Retrospective study.

Methods: Clinical examination findings and multimodal imaging features, including color fundus photography, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence, fluorescein and indocyanine green angiography (ICGA), and optical coherence tomography angiography (OCTA) images were evaluated retrospectively.

Results: We assessed 27 eyes of 16 patients with the diagnosis of Best disease. Only patients presenting with serous macular detachment and subretinal pigment epithelium hyperreflective lesion in one or both eyes were included in this study. In 17 of 27 eyes (63%), fibrosis was identified by multimodal imaging techniques. Although there was no sign of active neovascularization on fundus examination or SD-OCT, a vascular network could be identified in 7 eyes (26%) (in 1 eye with OCTA only and in 6 eyes with both OCTA and ICGA). Active neovascularization was seen in 3 eyes (11%). Treatment was recommended for eyes with active neovascularization, and follow-up was scheduled for eyes with quiescent neovascularization and fibrosis.

Conclusion: Eyes with Best disease with subretinal pigment epithelium hyperreflective lesion and serous macular detachment may show fibrosis, quiescent neovascularization, or active neovascularization. Multimodal imaging techniques are very important for differentiation of these lesions.

Keywords: Best disease; optical coherence tomography; optical coherence tomography angiography; quiescent neovascularization; subretinal pigment epithelium hyperreflective lesion.

MeSH terms

  • Epithelium / pathology
  • Fibrosis
  • Fluorescein Angiography / methods
  • Humans
  • Multimodal Imaging
  • Retinal Detachment* / diagnosis
  • Retinal Detachment* / pathology
  • Retinal Pigment Epithelium / pathology
  • Retrospective Studies
  • Tomography, Optical Coherence / methods
  • Visual Acuity
  • Vitelliform Macular Dystrophy* / pathology