FLUORESCENCE LIFETIME PATTERNS IN MACULAR TELANGIECTASIA TYPE 2.

Abstract

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. 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. All 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. 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 Tm of 22% over 2.1 years in the temporal fovea. 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.