Foveomacular Vitelliform Dystrophy Research Articles

Test performance of optical coherence tomography angiography in detecting retinal diseases: a systematic review and meta-analysis.

To investigate the diagnostic accuracy of optical coherence tomography angiography (OCTA) in detecting vascular characteristics of chorio-retinal disease. Evidence acquisition: We searched Web of Science, Scopus, and Medline by the citation of references and complemented these electronic searches by checking the list of references of included and review articles. Screening, selection, assessment, and extraction was performed in parallel by two authors. Evidence synthesis: Systematic review and exploratory meta-analysis. The ten studies that contributed to the meta-analysis enrolled 440 eyes and allowed constructing ten two-by-two tables. The tables reported on detection of choroidal neovascularization (CNV) in eyes suffering from either age-related macular degeneration (4), central serous chorioretinopathy (2), myopia (2), foveomacular vitelliform dystrophy (1), or a mixed cohort suffering from multiple retinal diseases (1). Of the ten studies, six used a cohort and four a case-control design. We found a pooled sensitivity of 0.90 (95% confidence intervals (CIs): 0.82-0.95) and a pooled specificity of 0.97 (95% CI: 0.89-0.99). Corresponding positive and negative likelihood ratios were 32.3 (95% CI: 7.4-141.6) and 0.10 (95% CI: 0.06-0.20), respectively. No pooling was possible for retinal vascular parameters of diabetic retinopathy, polypoidal choroidal vasculopathy, or detection of CNV activity. The results of highly biased and heterogeneous studies assessing the diagnostic performance of OCTA highlight the need for further analyses of methodologically sound and sufficiently sized clinical evaluations.

Bilateral choroidal neovascularisation associated with adult‐onset foveomacular vitelliform dystrophy

Adult‐onset foveomacular vitelliform dystrophy (AFVD) is an increasingly well‐recognised maculopathy, originally described by Gass1974 as ‘peculiar foveomacular dystrophy’ and characterised by bila...

Foveomacular Vitelliform Dystrophy, Adult Type: A Clinicopathologic Study Including Electron Microscopic Observations

Foveomacular vitelliform dystrophy, adult type (FVDAT), is a dystrophy of the retinal pigment epithelium (RPE) characterized in part by autosomal dominant inheritance, midlife onset, mild visual loss, and various patterns of foveal yellow deposits located at the level of the pigment epithelium. Detailed pathologic studies of two postmortem eyes from a 61-year-old woman with documented FVDAT disclosed striking abnormalities involving mainly the foveal RPE and overlying photoreceptors. By light microscopy, marked focal atrophy of the pigment epithelial cells in the foveolar area was bordered by hypertrophic RPE. Interposed between the atrophic RPE and Bruch's membrane were scattered eosinophilic, fusiform collagenous plaques. The sensory retina overlying the deranged RPE displayed marked atrophy of the outer nuclear layer with loss of photoreceptor inner and outer segments. Pigment-laden macrophages containing periodic acid -Schiff-positive material had migrated into the atrophic, outer sensory retina. Ultraviolet fluorescent microscopy demonstrated massive accumulation of lipofuscin pigment within the macular RPE as well as within macrophages in the atrophic outer retina. By scanning electron microscopy, a confluent area of flattened, atrophic retinal pigment epithelial cells was rimmed by taller, hypertrophic RPE cells..By transmission electron microscopy, the pigment epithelial cells contained myriad lipofuscin granules. Bruch's membrane and the intercapillary pillars were slightly thickened and the choriocapillaris was patent. The possible interrelationships between FVDAT and the other pattern dystrophies of the RPE are discussed.