Demographic features of idiopathic macular telangiectasia in Japanese patients

Maruko, Ichiro; Iida, Tomohiro; Sugano, Yukinori; Ojima, Akira; Oyamada, Hiroshi; Sekiryu, Tetsuju

doi:10.1007/s10384-011-0112-5

Cited by 14 publications

(17 citation statements)

References 20 publications

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“…It is less prevalent than MacTel type 2, although this might not be true in all populations (Lee et al, 2011; Maruko et al, 2012). Little is known of its pathophysiology and natural history.…”

Section: Differential Diagnosismentioning

confidence: 92%

Macular telangiectasia type 2

Issa

Gillies

Chew

et al. 2013

Progress in Retinal and Eye Research

328

244

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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 nonneovascular 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.

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Section: Differential Diagnosismentioning

confidence: 92%

Macular telangiectasia type 2

Issa

Gillies

Chew

et al. 2013

Progress in Retinal and Eye Research

328

244

View full text Add to dashboard Cite

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“…This study also shows the prominence of macular thinning among patients with XLAS, but it should not be considered pathognomonic because temporal macular thinning has also been reported in patients with proliferative sickle cell retinopathy and idiopathic macular telangiectasia. 32,33 The much lower number of patients with individual mutations other than L1649R represents a limitation to our study that precludes extensive statistical analyses.…”

Section: Commentmentioning

confidence: 99%

Temporal Macular Thinning Associated With X-Linked Alport Syndrome

Ahmed¹,

Kamae²,

Jones³

et al. 2013

JAMA Ophthalmol

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“…As the Mactel is a rare disease, the strategy of its treatment has not been established yet. Most instances of telangiectasia in Western countries are type 2, while the most frequent among the Japanese has been reported to be type 1 MacTel [4,5]. Laser photocoagulation, photodynamic therapy, and anti-VEGF agents have been applied for the treatment of type 1 MacTel.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of Laser Photocoagulation for Type 1 Idiopathic Macular Telangiectasia: A Retrospective Study

Kono

Yokoyama

Kishi

et al. 2021

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Aims: The aim of this study was to evaluate the efficacy of laser photocoagulation for type 1 idiopathic macular telangiectasia (MacTel). Study Design: Retrospective case series Place and Duration of Study: Department of Ophthalmology, Oita University Hospital during the period from 2004 to 2013. Patients and Methods: Six eyes of 6 patients with type 1 MacTel diagnosed consecutively in the Oita University Hospital during the period from 2004 to 2013 were studied. They showed macular edema and their visual acuity were deteriorated due to leakage from the aneurysms. Four patients were male and two were female with an average age of 64 years (range: 41 to 74 years). Informed consent was obtained from each patient and the laser photocoagulation was applied to leaky aneurysms in all eyes. The central macular thickness (CMT) was measured by optical coherence tomography (OCT). Mean follow up period was 44 months (range: 24 - 63 months). Results: Mean visual acuity significantly increased from logMAR 0.25±0.27 at baseline to logMAR 0.11±0.14 (P<0.05) at the last visit. Visual acuity improved more than 0.2 logMAR in five eyes and was stable (gain or loss of < 0.3 logMAR) in one eye. Mean central macular thickness significantly decreased from 454±181 μm at baseline to 231±67 μm (P<0.05) at the last visit. Macular edema was not detected by OCT inany patients at the last visit. Conclusion: In type I MacTel, laser photocoagulation may achieve a visual improvement and normalize the central macular thickness.

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Demographic features of idiopathic macular telangiectasia in Japanese patients

Abstract: The most frequent among our patients was aneurysmal MacTel, whereas perifoveal MacTel was not common. Although the clinical characteristics were similar, the most common type was different from that in Western countries.

Cited by 14 publications

References 20 publications

Macular telangiectasia type 2

Macular telangiectasia type 2

Temporal Macular Thinning Associated With X-Linked Alport Syndrome

Efficacy of Laser Photocoagulation for Type 1 Idiopathic Macular Telangiectasia: A Retrospective Study

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