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Management of Idiopathic Macular Telangiectasia Type 2.

ABSTRACT: Macular telangiectasia type 2 (MacTel) is a relatively rare disease without established treatments. Although MacTel was previously considered a primarily vascular condition, the thinking on its pathogenesis has shifted to it now being considered principally a neurodegenerative disease. This has resulted in a subsequent change in the approach to treatment toward neuro-protection for the non-proliferative phase of this disease. Carotenoid supplementation has had mixed results. Ciliary neurotrophic factor (CNTF) has demonstrated some promising early results, but further study is necessary to determine its actual effect. Some structural improvements have been seen in the non-proliferative phase with oral acetazolamide but without accompanying functional improvement. Anti-vascular endothelial drugs have been studied and not found to have benefit in the non-proliferative phase of disease but have demonstrated significant structural and functional value in the treatment of secondary neovascularization. There is no level I evidence for the various proposed MacTel treatments, and efforts need to be directed toward conducting multicenter randomized trials to better understand possible treatments for this condition.

SUBMITTER: Khodabande A

PROVIDER: S-EPMC6514016 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Management of Idiopathic Macular Telangiectasia Type 2.

Khodabande Alireza A Roohipoor Ramak R Zamani Javad J Mirghorbani Masoud M Zolfaghari Hamidreza H Karami Shahab S Modjtahedi Bobeck S BS

Ophthalmology and therapy 20190220 2

Macular telangiectasia type 2 (MacTel) is a relatively rare disease without established treatments. Although MacTel was previously considered a primarily vascular condition, the thinking on its pathogenesis has shifted to it now being considered principally a neurodegenerative disease. This has resulted in a subsequent change in the approach to treatment toward neuro-protection for the non-proliferative phase of this disease. Carotenoid supplementation has had mixed results. Ciliary neurotrophic ...[more]

PMID: 30788805

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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:PurposeType 2 idiopathic macular telangiectasia (MacTel) is a rare bilateral neurodegenerative disease characterized by alterations in the macular capillary network leading to central vision loss. The purpose of this study was to quantify disease-specific retinal fluorescence lifetime patterns in patients with MacTel using fluorescence lifetime imaging ophthalmoscopy.ParticipantsBoth eyes of 14 patients (mean age ± SEM, 67.8 ± 6.4 years) with a clinical diagnosis of MacTel Type 2 and 14 healthy age-matched controls (age 69.8 ± 6.4 years) were included in this study.MethodsAll participants were imaged with a fluorescence lifetime imaging ophthalmoscope (Heidelberg Engineering, Germany). Mean retinal fluorescence lifetimes (Tm) were obtained in the short spectral channels (498-560 nm) and long spectral channels (560-720 nm). Clinical features, fundus images, fundus autofluorescence intensity images, spectral domain optical coherence tomography, and corresponding macular pigment optical density measurements using a modified confocal scanning laser ophthalmoscope (mpHRA) were further analyzed. Patients were classified into five phenotypic subgroups using the Gass and Blodi classification.ResultsMean fluorescence lifetimes were significantly prolonged temporal to the fovea in patients with MacTel compared with healthy controls (mean ± SEM: short spectral channels 543 ± 61 ps vs. 304 ± 9 ps; P < 0.0001; long spectral channels: 447 ± 26 ps vs. 348 ± 11 ps; P < 0.0001), and appeared as a crescent or ring-shaped pattern. Prolonged lifetime patterns correlated with decreased macular pigment density on macular pigment optical density measurements. Follow-up examinations were performed in four MacTel patients, which revealed an increase of short spectral channel Tm of 22% over 2.1 years in the temporal fovea.ConclusionThis study confirms that fundus autofluorescence lifetimes display characteristic patterns in patients with MacTel Type 2 disease and provide information about macular pigment and possibly photoreceptor loss. Fluorescence lifetime prolongation correlates with disease severity and may therefore be a useful addition to other imaging modalities for assessing disease progression in MacTel Type 2.

Dataset Information

Management of Idiopathic Macular Telangiectasia Type 2.

Publications

Management of Idiopathic Macular Telangiectasia Type 2.

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