Foveal Avascular Zone in Idiopathic Juxtafoveolar Telangiectasia

Mansour, Ahmad M.; Schachat, Andrew P.

doi:10.1159/000310399

Cited by 22 publications

(16 citation statements)

References 11 publications

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“…A smaller foveal avascular zone compared to normal has been reported in patient with idiopathic juxtafoveolar telangiectasia (Mansour and Schachat, 1993); however, the proportion of patients with MacTel type 2 in this study remains unclear and it appears that mainly other disease entities had been analyzed. Another study described two cases with a centripetal retinal capillary proliferation (Koizumi et al, 2007a).…”

Section: Additional Associated Ocular Findings and Systemic Diseasesmentioning

confidence: 65%

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: Additional Associated Ocular Findings and Systemic Diseasesmentioning

confidence: 65%

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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“…A slight graying of the parafoveolar retina approximately one disc diameter in size, confined temporally or forming a partial or complete horizontal oval around the foveal center exists 1–318. The foveal center is spared and may appear thinned [Figure 3A].…”

Section: Group Iia: Occult and Nonexudative Idiopathic Juxtafoveolar mentioning

confidence: 99%

Idiopathic juxtafoveolar retinal telangiectasis: A current review

Nowilaty

Al-Shamsi

Al-Khars

2010

Middle East Afr J Ophthalmol

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Idiopathic juxtafoveolar retinal telangiectasis (IJFT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities.

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“…The FAZ was reported small in hereditary posterior microphtalmos [1], retinopathy of prematurity [2], and idiopathic juxtafoveolar telangectasia [3], and enlarged in diabetic retinopathy [4][5][6][7], sickle cell retinopathy [8][9][10][11][12], talc retinopathy [ 13], and branch retinal vein occlusion [ 14]. In this study we attempted to evaluate the modification of area and perimeter of the FAZ in patients with macular branch retinal vein occlusion (MBRVO).…”

Section: Introductionmentioning

confidence: 99%

Foveal avascular zone in macular branch retinal vein occlusion

et al. 1995

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The mean area of the foveal avascular zone (FAZ) in normal subjects was reported as 0.231 mm2 to 0.405 mm2, using fluorescein angiography. The FAZ enlarges in vaso-occlusive diseases, especially diabetic retinopathy, sickle cell retinopathy, talc retinopathy and branch retinal vein occlusion. In the present study the FAZ of 20 patients affected by macular branch retinal vein occlusion (MBRVO) was compared with the FAZ of 41 control subjects. The FAZ mean area was 0.56 +/- 0.34 mm2 SD in the MBRVO group, while 0.26 +/- 0.07 mm2 SD in the control group, with a statistically significant difference (p < 0.001). The FAZ mean perimeter was 4.77 +/- 1.90 mm SD in the MBRVO group, and 2.36 +/- 0.32 mm SD in the control group, with a statistically significant difference (p < 0.001). Taking into account the MBRVO group, a statistical correlation was found between visual acuity impairment and FAZ enlargement (p = 0.02), but not between visual acuity impairment and macular edema (p = 0.41). In 14 cases (70%) secondary avascular microzones located in the macular sector correspondent to MBRVO were also evident. MBRVO causes an irregular enlargement of the FAZ which seems to represent the most important feature related to visual acuity impairment.

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Foveal Avascular Zone in Idiopathic Juxtafoveolar Telangiectasia

Cited by 22 publications

References 11 publications

Macular telangiectasia type 2

Macular telangiectasia type 2

Idiopathic juxtafoveolar retinal telangiectasis: A current review

Foveal avascular zone in macular branch retinal vein occlusion

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