title: Multimodal imaging in type 2 idiopathic macular telangiectasia.
abstract: BACKGROUND
  Macular telangiectasia Type 2 is a bilateral, progressive potentially blinding retinal disease characterized by both vascular and neurodegenerative signs that have been documented using different imaging techniques. The correlation between macular telangiectasia Type 2 signs from various imaging modalities is unknown. Our aim was to investigate the relationship of various macular telangiectasia Type 2 signs using fundus fluorescein angiography, optical coherence tomography and dual-wavelength autofluorescence images.
  
  
  METHODS
  Participants were selected from the macular telangiectasia Type 2 Natural History Observation Study, based on a confirmed diagnosis and the availability of images. Signs in fundus fluorescein angiography, dual-wavelength autofluorescence, and optical coherence tomography images were graded according to standardized categories, and agreement among the multimodel imaging was assessed statistically.
  
  
  RESULTS
  One hundred and ninety-one eyes of 96 patients were examined. Significant correlations were found between early and late fundus fluorescein angiography (ρ = 0.82, P < 0.0001), luteal pigment loss and early/late fundus fluorescein angiography signs (ρ = 0.52, P < 0.0001 and ρ = 0.62, P < 0.0001, respectively), inner and outer segment break length and pigment loss (Class 1 vs. 2/3, P < 0.0001; Class 2 vs. 3, P = 0.04). Correlation between pigment loss and retinal spaces/atrophic retinal restructuring was fair (κ = 0.25-0.33). Bilateral symmetry was slight to substantial (κ = 0.18-0.62).
  
  
  CONCLUSIONS
  Our data demonstrate the relative extent of neurodegenerative and vascular signs; it may be useful for designing systems for staging disease severity using multimodal imaging and may also provide clues to the pathogenesis of the disease.
authors.affiliation: *Department of Research and Development, Moorfields Eye Hospital, London, United Kingdom; †UCL Institute of Ophthalmology, London, United Kingdom; ‡The EMMES Corporation, Rockville, Maryland; §NIHR Biomedical Research Center for Ophthalmology, Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, United Kingdom; ¶Inherited Eye Disease, Moorfields Eye Hospital, London, United Kingdom; **Department of Ophthalmology, St. Franziskus Hospital, Münster, Germany.
authors.forename: Ferenc B
authors.initials: FB
authors.lastname: Sallo
authors.forename: Irene
authors.initials: I
authors.lastname: Leung
authors.forename: Traci E
authors.initials: TE
authors.lastname: Clemons
authors.forename: Tunde
authors.initials: T
authors.lastname: Peto
authors.forename: Alan C
authors.initials: AC
authors.lastname: Bird
authors.forename: Daniel
authors.initials: D
authors.lastname: Pauleikhoff
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D061848:Optical Imaging; D010641:Phenotype; D011446:Prospective Studies; D058456:Retinal Telangiectasis / Q000175:diagnosis*; D012171:Retinal Vessels / Q000473:pathology; D041623:Tomography, Optical Coherence