Retinal pigment epithelium aperture: A late-onset complication in adult-onset foveomacular vitelliform dystrophy

Abstract

Purpose: The purpose of the study was to report aperture of retinal pigment epithelium (RPE) as a late complication and an unreported finding during the natural course of adult-onset foveomacular vitelliform dystrophy (AFVD). Methods: Four diagnosed cases of AFVD followed for a period ranging from 4 to 8 years. All patients had documented records of clinical examination, fundus autofluorescence and fluorescein angiography, and spectral domain-optical coherence tomography at regular intervals. Results: Besides the known stages in the natural course of AFVD, RPE aperture was noted as an additional finding during the vitelliruptive stage of the disease. The vitelliform material was noted beneath the disrupted RPE before disappearance. Accumulation of vitelliform material continued even after the vitelliruptive stage. Conclusion: RPE aperture may represent an ongoing process in the natural course of AFVD.

Keywords

• Adult-onset foveomacular vitelliform dystrophy
• aperture
• fundus autofluorescence
• retinal pigment epithelium
• spectral domain-optical coherence tomography