Optical coherence tomography findings in adult-onset foveomacular vitelliform dystrophy

Abstract

PURPOSE: The purpose of this article is to analyze cross-sectional images of a subretinal macular lesion, using optical coherence tomography (OCT) in eyes with adult-onset foveomacular vitelliform dystrophy (AOFVD), to compare thickness of the neurosensory retina over the lesion with best-corrected visual acuity (BCVA) of each eye, and to compare OCT tomograms of AOFVD patients with OCT of Best disease. DESIGN: Observational case series. METHODS: This is a retrospective study which took place in a clinical practice. Forty-three patients (72 eyes) with AOFVD and 12 patients (24 eyes) with Best’s disease were studied. The observation procedures used were biomicroscopic fundus examination, fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). The main outcome measures were a description of the typical picture of AOFVD in OCT tomograms, the relationship between the neurosensory retinal thickness over the lesion, the BCVA expressed in decimal terms, and a comparison with description of OCT in Best disease. RESULTS: Of the 43 patients affected by AOFVD, 29 had bilateral macular lesions. Fluorescein angiography showed a central hypofluorescent spot surrounded by an irregular hyperfluorescent ring in 65 of the 72 eyes. Indocyanine green angiography demonstrated a central nonfluorescent spot and a hyperfluorescent area surrounding the central spot in 22 of 27 eyes examined. In all 72 eyes of 43 patients, OCT showed a well-defined central region of thickening in the reflective band representing the retinal pigment epithelium (RPE). The relationship between the thickness of neurosensory retina over the lesion and BCVA was significant ( P = .001, r 2 = 0.61). Optical coherence tomography in all 24 eyes with Best disease showed a well-defined central serous retinal detachment. CONCLUSION: In the 72 eyes with AOFVD, FA and ICGA presented different features. Instead, OCT tomograms showed a well-defined subretinal thickening of the RPE in all the eyes. The lack of difference in OCT patterns between cases with or without the hypofluorescent spot on angiography was useful for confirming the diagnosis of AOFVD. Moreover, a reduced visual acuity was evident in patients with a thinner neurosensory retinal layer over AOFVD lesion. Finally, OCT images were also useful for distinguishing AOFVD from Best disease.