CORRELATION OF OPTICAL COHERENCE TOMOGRAPHY AND MACULAR PIGMENT OPTICAL DENSITY MEASUREMENTS IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA.

PURPOSE: Macular telangiectasia is associated with neurodegenerative changes including focal outer retinal atrophy and a loss of macular pigment (MP). We aimed to investigate whether an association between spectral domain optical coherence tomography neurodegenerative signs and MP abnormalities can be detected. METHODS: Forty-seven eyes of 27 macular telangiectasia Type 2 patients (mean age 66.7 years, range 50-82 years, 12 male) were investigated. An MP pattern was recorded using a dual-wavelength autofluorescence method and classified according to severity (I-III). Outer plexiform, inner nuclear, and photoreceptor layer thickness values were measured in Spectralis spectral domain optical coherence tomography scans. Thickness values were compared with those of a control group of 14 healthy age-matched eyes. RESULTS: Macular pigment redistribution was found to be Class I in 11 eyes, Class II in 28 eyes, and Class III in 8 eyes. More advanced stages of MP loss were associated with a greater, statistically significant thinning of the outer plexiform and inner nuclear layer complex and photoreceptor layers (P ≤ 0.001). Lower absolute levels of MP were also associated with a thinning of the photoreceptor layer. Thinning was restricted to within the parafovea, more severe at temporal eccentricities. CONCLUSION: Our findings support the hypothesis that in macular telangiectasia Type 2 cellular degenerative processes leading to a thinning of these layers also result in reduction and redistribution of MP.

Macular telangiectasia--changes in macular pigment optical density during a 5-year follow-up.

PURPOSE: To quantitatively analyze the distribution of macular pigment (MP) over a period of 5 years and for monitoring progression of macular telangiectasia. METHODS: Macular pigment concentration (autofluorescence, excitation wavelengths: 488 and 514 nm) was determined at baseline and after 5 years in 43 eyes of 22 subjects (46-80 years; mean, 65.6 years; 10 men) participating in the macular telangiectasia project. RESULTS: Mean MP density at 0.5° declined in the segment (one eighth of a circle) with the highest MP optical density (-0.04 density units; P= 0.015), where density units (DU), and also averaged in the 2 segments that divided segments with detectable MP from those in which MP was no longer detectable (-0.04 density units; P = 0.0005). In the first segment mentioned, 2° values decreased to a lesser extent and not significantly. The diameter of MP loss expanded horizontally from 2.64 mm to 2.74 mm (P = 0.0001) but not vertically. Macular pigment density in the "halo" of peripheral MP at a mean of 5.44° (4.53-6.21°) increased (+0.01 DU; P= 0.01). CONCLUSION: Five years of follow-up resulted in central (0.5°) reduction and peripheral (4.53-6.21°) accumulation of MP. Longer period of follow-up may disclose significant changes in paracentral locations. The area of central MP loss expands in particular in a horizontal direction and less vertically. Centrifugal movement of MP during disease may explain our findings.

Macular telangiectasia: patterns of distribution of macular pigment and response to supplementation.

PURPOSE: By analyzing the patterns of macular pigment (MP) in type 2 idiopathic telangiectasia eyes, different stages in the changes reflecting the extent of disease can be observed. The purpose of this study was to determine whether the amount and the pattern of MP can be influenced by supplementation. METHOD: Eleven patients with type 2 idiopathic telangiectasia received 12 mg lutein and 0.6 mg zeaxanthin (Ocuvite Lutein AMD) daily for 9 months. For a period of 12 months, MP concentration was determined every 3 months by autofluorescence (2 excitation wavelengths: 488 and 514 nm). RESULTS: When central accumulation of MP was similar to that in healthy subjects (with segment of reduced MP in the temporal fovea: MP Class I), supplementation enriched the MP at 0.5 degrees, 2 degrees, and 5 degrees to 6 degrees. In MP Class II (reduced concentration of MP centrally), accumulation could be detected at 2 degrees and 5 degrees to 6 degrees but not centrally. In MP Class III (oval-shaped effacement of MP centrally, surrounding oval-shaped ring of MP at 5 degrees-7 degrees eccentricity), supplementation promoted MP accumulation only at 5 degrees to 6 degrees. CONCLUSION: After oral supplementation with lutein/zeaxanthin, an increase in the MP was detected only in areas where the MP was present at baseline. Supplementation did not produce an increase in the area where the MP was absent. Degenerative processes causing an impairment in transport and storage of lutein and zeaxanthin may play a leading role in the pathogenesis of type 2 idiopathic telangiectasia.

Idiopathic macular telangiectasia type 2: distribution of macular pigment and functional investigations.

PURPOSE: Analysis and categorization of macular pigment (MP) distribution in type 2 idiopathic macular telangiectasia (IMT2) with regard to a possible grading scale for the severity of the disease. METHODS: Nineteen IMT2 patients were examined including visual acuity (VA), fundus biomicroscopy, fluorescein angiography (FLA), microperimetry and optical coherence tomography (OCT). Distribution of MP was analyzed and categorized in MP density maps calculated from autofluorescence images obtained at 488 and 514 nm excitation wavelengths. RESULTS: Typical features in MP density maps are in class I (n=8), a triangular segment of reduced MP in the temporal fovea and central accumulation of MP, class II (n=12), further expansion of the segment and vanishing of central accumulation, and class III (n=18), oval effacement of MP centrally, surrounding halo of MP at 5-7 degrees eccentricity. These classes were associated with the stages of the disease and increasing restrictions in visual function. CONCLUSION: Association between changes in MP distribution, stages of IMT2 and restrictions in visual functions suggests that the classification of MP patterns reflects a severity scale for IMT2. Degenerative processes causing impairments in transport and storage of lutein (L) and zeaxanthin (Z) leading to secondary vascular changes may play a causative role in the disease.