title: CONE DENSITY LOSS ON ADAPTIVE OPTICS IN EARLY MACULAR TELANGIECTASIA TYPE 2.
abstract: OBJECTIVE
  To determine whether cone photoreceptors are impaired early in macular telangiectasia type 2 (MacTel 2) progression.
  
  
  METHODS
  Eight patients with MacTel 2 lacking intraretinal cavitation underwent multimodal retinal imaging including adaptive optics. Cone packing metrics were determined in 5 sampling windows at different eccentricities from the fovea. Results were compared with a previously established normative database.
  
  
  RESULTS
  In MacTel 2 eyes, cone density was significantly lower than normal at all eccentricities (P < 0.0001). Mean cone spacing and mean percentage of hexagonally organized cone photoreceptors were respectively significantly larger and lower than normal at all eccentricities (P = 0.0488 and P < 0.0001). In MacTel 2 patients, adaptive optics showed an irregular patchy disturbance of the cone mosaic corresponding to some fragmentation of the interdigitation zone on optical coherence tomography. The ellipsoid zone remained intact in the studied area.
  
  
  CONCLUSIONS
  Adaptive optics showed that the macular cone density was lower than normal even outside the telangiectasia in MacTel 2 lacking intraretinal cavitation, although the ellipsoid zone remained intact on optical coherence tomography. These findings do not indicate that the cone density loss is causative of the disease as it might be secondary to Müller cell or rod loss in this area. However, cone density assessment could become a useful parameter to monitor disease progression.
authors.affiliation: *Assistance Publique-Hôpitaux de Paris AP-HP, Hôpital Lariboisière, Department of Ophthalmology, Université Paris Diderot, Sorbonne Paris Cité, Paris, France; †Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium; and ‡Clinical Investigation Center 1423, Centre Hospitalier National des Quinze-Vingts, Institut National de la Santé et de la Recherche Médicale & Université Pierre et Marie Curie, Paris, France.
authors.forename: Julie
authors.initials: J
authors.lastname: Jacob
authors.forename: Valérie
authors.initials: V
authors.lastname: Krivosic
authors.forename: Michel
authors.initials: M
authors.lastname: Paques
authors.forename: Ramin
authors.initials: R
authors.lastname: Tadayoni
authors.forename: Alain
authors.initials: A
authors.lastname: Gaudric
mesh_terms: D000368:Aged; D002452:Cell Count; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D009887:Ophthalmoscopy; D017949:Retinal Cone Photoreceptor Cells / Q000473:pathology*; D058456:Retinal Telangiectasis / Q000145:classification / Q000175:diagnosis* / Q000503:physiopathology; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology