Dataset Information

ABNORMAL RETINAL REFLECTIVITY TO SHORT-WAVELENGTH LIGHT IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA.

ABSTRACT: PURPOSE:Macular telangiectasia Type 2 (MacTel) is a bilateral, progressive, potentially blinding retinal disease characterized by vascular and neurodegenerative signs, including an increased parafoveal reflectivity to blue light. Our aim was to investigate the relationship of this sign with other signs of macular telangiectasia Type 2 in multiple imaging modalities. METHODS:Participants were selected from the MacTel Type 2 study, based on a confirmed diagnosis and the availability of images. The extent of signs in blue-light reflectance, fluorescein angiographic, optical coherence tomographic, and single- and dual-wavelength autofluorescence images were analyzed. RESULTS:A well-defined abnormality of the perifovea is demonstrated by dual-wavelength autofluorescence and blue-light reflectance in early disease. The agreement in area size of the abnormalities in dual-wavelength autofluorescence and in blue-light reflectance images was excellent: for right eyes: ? = 0.917 (P < 0.0001, 95% confidence interval 0.855-0.954, n = 46) and for left eyes: ? = 0.952 (P < 0.0001, 95% confidence interval 0.916-0.973, n = 49). Other changes are less extensive initially and expand later to occupy that area and do not extend beyond it. CONCLUSION:Our findings indicate that abnormal metabolic handling of luteal pigment and physical changes giving rise to increased reflectance are widespread in the macula throughout the natural history of the disease, precede other changes, and are relevant to early diagnosis.

SUBMITTER: Sallo FB

PROVIDER: S-EPMC5726908 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature

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ABNORMAL RETINAL REFLECTIVITY TO SHORT-WAVELENGTH LIGHT IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA.

Sallo Ferenc B FB Leung Irene I Zeimer Meike M Clemons Traci E TE Dubis Adam M AM Fruttiger Marcus M Pauleikhoff Daniel D Chew Emily Y EY Egan Catherine C Peto Tunde T Bird Alan C AC

Retina (Philadelphia, Pa.) 20180101

<h4>Purpose</h4>Macular telangiectasia Type 2 (MacTel) is a bilateral, progressive, potentially blinding retinal disease characterized by vascular and neurodegenerative signs, including an increased parafoveal reflectivity to blue light. Our aim was to investigate the relationship of this sign with other signs of macular telangiectasia Type 2 in multiple imaging modalities.<h4>Methods</h4>Participants were selected from the MacTel Type 2 study, based on a confirmed diagnosis and the availability ...[more]

PMID: 28644304

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Project description:Macular telangiectasia type 2 is a bilateral disease of unknown cause with characteristic alterations of the macular capillary network and neurosensory atrophy. Its prevalence may be underestimated and has recently been shown to be as high as 0.1% in persons 40 years and older. Biomicroscopy may show reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy, and neovascular complexes. Fluorescein angiography shows telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. High-resolution optical coherence tomography (OCT) may reveal disruption of the photoreceptor inner segment-outer segment border, hyporeflective cavities at the level of the inner or outer retina, and atrophy of the retina in later stages. Macular telangiectasia type 2 shows a unique depletion of the macular pigment in the central retina and recent therapeutic trials showed that such depleted areas cannot re-accumulate lutein and zeaxanthin after oral supplementation. There have been various therapeutic approaches with limited or no efficacy. Recent clinical trials with compounds that block vascular endothelial growth factor (VEGF) have established the role of VEGF in the pathophysiology of the disease, but have not shown significant efficacy, at least for the non-neovascular disease stages. Recent progress in structure-function correlation may help to develop surrogate outcome measures for future clinical trials. In this review article, we summarize the current knowledge on macular telangiectasia type 2, including the epidemiology, the genetics, the clinical findings, the staging and the differential diagnosis of the disease. Findings using retinal imaging are discussed, including fluorescein angiography, OCT, adaptive optics imaging, confocal scanning laser ophthalmoscopy, and fundus autofluorescence, as are the findings using visual function testing including visual acuity and fundus-controlled microperimetry. We provide an overview of the therapeutic approaches for both non-neovascular and neovascular disease stages and provide a perspective of future directions including animal models and potential therapeutic approaches.

Project description:ImportanceAs currently used, microperimetry is a burdensome clinical testing modality for testing retinal sensitivity requiring long testing times and trained technicians.ObjectiveTo create a deep-learning network that could directly estimate function from structure de novo to provide an en face high-resolution map of estimated retinal sensitivity.Design, setting, and participantsA cross-sectional imaging study using data collected between January 1, 2016, and November 30, 2017, from the Natural History Observation and Registry of macular telangiectasia type 2 (MacTel) evaluated 38 participants with confirmed MacTel from 2 centers.Main outcomes and measuresMean absolute error of estimated compared with observed retinal sensitivity. Observed retinal sensitivity was obtained with fundus-controlled perimetry (microperimetry). Estimates of retinal sensitivity were made with deep-learning models that learned on superpositions of high-resolution optical coherence tomography (OCT) scans and microperimetry results. Those predictions were used to create high-density en face sensitivity maps of the macula. Training, validation, and test sets were segregated at the patient level.ResultsA total of 2499 microperimetry sensitivities were mapped onto 1708 OCT B-scans from 63 eyes of 38 patients (mean [SD] age, 74.3 [9.7] years; 15 men [39.5%]). The numbers of examples for our algorithm were 67 899 (103 053 after data augmentation) for training, 1695 for validation, and 1212 for testing. Mean absolute error results were 4.51 dB (95% CI, 4.36-4.65 dB) when using linear regression and 3.66 dB (95% CI, 3.53-3.78 dB) when using the LeNet model. Using a 49.9 million-variable deep-learning model, a mean absolute error of 3.36 dB (95% CI, 3.25-3.48 dB) of retinal sensitivity for validation and test was achieved. Correlation showed a high degree of agreement (Pearson correlation r = 0.78). By paired Wilcoxon rank sum test, our model significantly outperformed these 2 baseline models (P < .001).Conclusions and relevanceHigh-resolution en face maps of estimated retinal sensitivities were created in eyes with MacTel. The maps were of unequalled resolution compared with microperimetry and were able to correctly delineate functionally healthy and impaired retina. This model may be useful to monitor structural and functional disease progression and has potential as an objective surrogate outcome measure in investigational trials.

Dataset Information

ABNORMAL RETINAL REFLECTIVITY TO SHORT-WAVELENGTH LIGHT IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA.

Publications

ABNORMAL RETINAL REFLECTIVITY TO SHORT-WAVELENGTH LIGHT IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA.

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