Retina. 2018 Jan;38 Suppl 1(Suppl 1):S43-S50. doi: 10.1097/IAE.0000000000001842.


Purpose: Pigment in the midretina is a characteristic sign in Type 2 idiopathic macular telangiectasia (MacTel) and is considered to characterize the late stage of the disease. Our aim was to investigate its incidence, and relationship with risk factors for MacTel, including outer retinal vascularization and subretinal neovascular proliferation (SRNV).

Methods: Pigment extent was measured in fundus autofluorescence images of 150 eyes of 75 MacTel probands, using the Region Finder tool of Heidelberg Eye Explorer. A linear mixed model was used to analyze the dynamics of pigment and its associations with other features of the phenotype. The relative incidence of pigment and of outer retinal outer retinal vascularization and SRNV was analyzed within the full MacTel Study cohort (1,244 probands).

Results: Mean pigment area at baseline was 0.157 mm (range = 0-1.295 mm, SD = 0.228 mm, n = 101). Progression demonstrated a nonlinear pattern (P < 0.001) at an overall rate of 0.0177 mm/year and was associated with the initial plaque size and with SRNV. There was a strong correlation between fellow eyes (P ≤ 0.0001). In approximately 25% of all SRNV cases, SRNV may coincide with or precede pigment.

Conclusion: Our data may be useful for refining the current system for staging disease severity in MacTel.

Publication types

  • Multicenter Study

MeSH terms

  • Female
  • Fluorescein Angiography / methods*
  • Follow-Up Studies
  • Fundus Oculi
  • Humans
  • Macula Lutea / metabolism*
  • Macula Lutea / pathology
  • Male
  • Middle Aged
  • Ophthalmoscopy / methods*
  • Prospective Studies
  • Retinal Pigment Epithelium / metabolism
  • Retinal Pigment Epithelium / pathology*
  • Retinal Pigments / metabolism*
  • Telangiectasia, Hereditary Hemorrhagic / diagnosis
  • Telangiectasia, Hereditary Hemorrhagic / metabolism*
  • Telangiectasia, Hereditary Hemorrhagic / physiopathology
  • Time Factors
  • Tomography, Optical Coherence / methods*
  • Visual Acuity*


  • Retinal Pigments

Supplementary concepts

  • Osler-rendu-weber syndrome 2