Fluorescence Lifetime Imaging Ophthalmoscopy: A Novel Way to Assess Macular Telangiectasia Type 2

Sauer, Lydia; Gensure, Rebekah H.; Hammer, Martin; Bernstein, Paul S.

doi:10.1016/j.oret.2017.10.008

Cited by 59 publications

(89 citation statements)

References 64 publications

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“…In our study of 28 eyes of patients with MacTel Type 2, unique features of mean retinal fluorescence lifetimes were identified, which are in keeping with a recently published report. 20 We observed significantly prolonged lifetimes within the affected MacTel area, with the inferotemporal region showing the most pronounced changes with a general prolongation even in the very early stages of this disease. In addition, MPOD measurements correlated well with FLIO measurements, with both methods showing a loss of macular pigment with progression of the disease.…”

Section: Discussionmentioning

confidence: 61%

“…In a recent study, MacTel-specific fluorescence lifetime patterns have been reported. 20 The purpose of this study was to confirm these findings, to quantify disease-specific lifetime patterns in patients with MacTel, and to provide longitudinal fluorescence lifetime data.…”

mentioning

confidence: 80%

“…[14][15][16][17] Because the diagnosis of MacTel can be challenging especially at early stages of disease, FLIO may serve as an additional tool for early detection of Mac-Tel and possibly obtain information on disease progression. Because FLIO has been shown to detect changes in macular pigment with high contrast [18][19][20] changes in macular pigment specific lifetimes provide information about macular photoreceptor or possibly Müller cell loss. In a recent study, MacTel-specific fluorescence lifetime patterns have been reported.…”

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confidence: 99%

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Fluorescence Lifetime Patterns in Macular Telangiectasia Type 2

Solberg

Dysli

Wolf

et al. 2020

Retina

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Purpose: Type 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. Participants: Both 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. Methods: All participants were imaged with a fluorescence lifetime imaging ophthalmoscope (Heidelberg Engineering, Germany). Mean retinal fluorescence lifetimes (Ƭm) 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. Results: Mean 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 Ƭ m of 22% over 2.1 years in the temporal fovea. Conclusion: This 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.

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Section: Discussionmentioning

confidence: 61%

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confidence: 80%

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confidence: 99%

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Fluorescence Lifetime Patterns in Macular Telangiectasia Type 2

Solberg

Dysli

Wolf

et al. 2020

Retina

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“…The theories and technical details behind FLIO have been described in more detail in previous publications. [9][10][11][12][13] Analysis of Fluorescence Lifetime Data…”

Section: Fluorescence Lifetime Imaging Ophthalmoscopementioning

confidence: 99%

“…8 Fluorescence lifetime imaging ophthalmoscopy (FLIO), an in vivo FLIM method, is a novel noninvasive imaging modality currently in clinical use to identify and quantify metabolic abnormalities in retinal diseases by imaging lifetimes of endogenous retinal fluorophores. [9][10][11][12][13] The fluorescence lifetime represents the time span an intrinsic fluorophore spends in a higher energy level following an excitation with a blue laser before returning to its ground level by releasing a photon with a longer emission wavelength.…”

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confidence: 99%

Fluorescence Lifetimes in Patients With Hydroxychloroquine Retinopathy

Solberg

Dysli

Möller

et al. 2019

Invest. Ophthalmol. Vis. Sci.

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PURPOSE. To investigate fundus autofluorescence lifetime features in patients with hydroxychloroquine (HCQ) retinopathy, and to identify early markers of retinal alterations in patients due to HCQ. METHODS. Patients attending screening for HCQ retinopathy were imaged with a fluorescence lifetime imaging ophthalmoscope. Mean retinal fluorescence lifetimes (Tm) were obtained in a short spectral channel (SSC, 498-560 nm) and a long spectral channel (LSC, 560-720 nm). Screening modalities included fundus images, fundus autofluorescence intensity images (FAF), spectral-domain optical coherence tomography (SD-OCT), visual fields, and multifocal electroretinogram (mfERG). RESULTS. Forty-two eyes of 21 patients on HCQ therapy and 40 eyes of 20 healthy age-matched controls were included. Fourteen eyes of 7 patients with HCQ retinopathy (mean age, 66.1 [SD, 7.7] years) and 28 eyes of 14 patients (mean age, 46.1 [SD, 7.9] years) receiving HCQ without retinopathy were identified. Patients with HCQ retinopathy showed a parafoveal ringshaped or oval area of prolonged mean fluorescence lifetimes. In these areas, mean (6SEM) lifetimes were 374 6 7 ps in the SSC, and on average 19.4% longer compared to the control group (P ¼ 0.0001). Patients on HCQ without retinopathy had retinal fluorescence lifetimes that were similar to the control group. CONCLUSIONS. This study shows that HCQ retinopathy displays characteristic mean fluorescence lifetimes.

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Genetic Penetrance of Macular Telangiectasia Type 2

et al. 2018

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acular telangiectasia type 2 (MacTel) was originally described by several researchers as an idiopathic juxtafoveal retinal telangiectasis. [1][2][3] MacTel is characterized by pigment clumping and telangiectatic vessels temporal to the macula, commonly with outer and inner temporal juxtafoveal cavitations and thinning. Juxtafoveal telangiectatic vessels are highlighted during the early stages of fluorescein angiography with temporal or perifoveal leakage and/or staining at later timeframes, and there is loss or redistribution of central macular pigment. [3][4][5] Increased confocal blue-light reflectance has also been demonstrated, but the mechanism of this phenomenon has not been elucidated. 5 There are several reports showing vertical transmission of disease suggestive of an autosomal dominant mode of inheritance, [6][7][8] but if this is the case, it is not fully penetrant because the siblings' prevalence of MacTel in these pedigrees is far below 50%. Although multiple genetic loci have been associated with MacTel, causative genetic mutations have yet to be identified. 9 To our knowledge, there are no published reports calculating a genetic penetrance estimate for MacTel. Accurately and precisely calculating genetic penetrance requires sufficiently large families with multiple affected individuals and rigorous phenotyping of as many family members as possible. Here we report a cross-sectional study of well-phenotyped MacTel family pedigrees from Utah and Idaho and calculate the apparent genetic penetrance of disease among the probands' parents and siblings. Methods Study DesignThis study was approved by the University of Utah institutional review board and complied with the Declaration of IMPORTANCE The apparent genetic penetrance of macular telangiectasia type 2 (MacTel) is important for gene discovery studies and for clinical risk assessment of affected individuals' family members. OBJECTIVE To determine the genetic penetrance of MacTel. DESIGN, SETTING, AND PARTICIPANTS Descriptive cross-sectional study of patients withMacTel at a tertiary referral eye center. From 2008 to 2016, consecutive patients with MacTel were independently identified, and all of their available siblings and parents were recruited. Seventeen probands with MacTel were included in the study who satisfied the requirement of having at least 1 parent or sibling willing and able to participate. Data from these 17 families were included for the analysis of apparent genetic penetrance. MAIN OUTCOMES AND MEASURES Determination of MacTel genetic penetrance in probands' parents and siblings.RESULTS Of 80 study participants, 50 (62.5%) were women. The mean (SD) age of study participants with MacTel was 61.2 (14.0) years (range, 23-81 years) and without MacTel was 60.7 (16.4) years (range, 24-92 years). There were 17 MacTel probands, and there was a high rate of enrollment of living siblings and parents: 52 of 71 living siblings (73%) and 11 of 12 parents (92%). Of 52 enrolled siblings, 9 (17%) were affected. Of 11 enrolled parents, 3 (27%) had Ma...

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Fluorescence Lifetime Imaging Ophthalmoscopy: A Novel Way to Assess Macular Telangiectasia Type 2

Cited by 59 publications

References 64 publications

Fluorescence Lifetime Patterns in Macular Telangiectasia Type 2

Fluorescence Lifetime Patterns in Macular Telangiectasia Type 2

Fluorescence Lifetimes in Patients With Hydroxychloroquine Retinopathy

Genetic Penetrance of Macular Telangiectasia Type 2

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