title: Ultrastructural and clinical evidence of subretinal debris accumulation in type 2 macular telangiectasia.
abstract: OBJECTIVE
  To describe subretinal debris found on ultrastructural examination in an eye with macular telangiectasia (MacTel) type 2 and on optical coherence tomography (OCT) in a subset of patients with MacTel type 2.
  
  
  METHODS
  Blocks from the mid-periphery and temporal perifovea of an eye with clinically documented MacTel type 2 were examined with electron microscopy (EM). Cases came from the Sydney centre of the MacTel project and the practices of the authors.
  
  
  RESULTS
  On EM examination, subretinal debris was found in the perifovea with accumulation of degenerate photoreceptor elements in the subretinal space. Despite the substantial subretinal debris, there was minimal retinal pigment epithelial (RPE) reaction. Focal defects were seen in the inner limiting membrane in the perifovea. Of the 65 Sydney MacTel project participants, three (5%) had prominent yellow material at the fovea. OCT revealed smooth mounds between the RPE and the ellipsoid region. The material was hyperautofluorescent.
  
  
  CONCLUSIONS
  This study suggests that subretinal accumulation of photoreceptor debris may be a feature of MacTel type 2. Ultrastructural and OCT evidence of disease beyond the vasculature, involving photoreceptors and Muller cells, is presented.
authors.affiliation: Anatomical Pathology, SEALS, Prince of Wales Hospital, Randwick, New South Wales, Australia.
authors.forename: Svetlana
authors.initials: S
authors.lastname: Cherepanoff
authors.forename: Murray C
authors.initials: MC
authors.lastname: Killingsworth
authors.forename: Meidong
authors.initials: M
authors.lastname: Zhu
authors.forename: Timothy
authors.initials: T
authors.lastname: Nolan
authors.forename: Alex P
authors.initials: AP
authors.lastname: Hunyor
authors.forename: Stephanie H
authors.initials: SH
authors.lastname: Young
authors.forename: Gregory S
authors.initials: GS
authors.lastname: Hageman
authors.forename: Mark C
authors.initials: MC
authors.lastname: Gillies
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D017949:Retinal Cone Photoreceptor Cells / Q000648:ultrastructure*; D017948:Retinal Rod Photoreceptor Cells / Q000648:ultrastructure*; D058456:Retinal Telangiectasis / Q000175:diagnosis*; D041623:Tomography, Optical Coherence / Q000379:methods*