Peripapillary Chorioretinal Atrophy (PPCRA) in normal eyes: Histopathology and photoreceptor loss

Academic Article


  • Purpose: The PP region features CRA in most normal eyes, prominent age-related changes in Bruch's membrane (BM), and a risk for choroidal neovascularization (CNV). We described the PP pigment epithelium (PE)/BM complex and photoreceptor distribution in eyes screened to exclude glaucoma, ≥4D myopia, and age-related macular degeneration (AMD). Methods: Eyes were obtained from 6 donors (64-93 yr) ≥ 2.5 hr post-mortem and 4 patients (77-87 yr) undergoing orbital exenteration (OE) for craniofacial tumors. The temporal side of the optic nerve head was sectioned for semi-quantitative light and electron microscopic histopathology. Five other OE retinas (60-77 yr) were whole mounted for photoreceptor counts. Results: All eyes had PP pigmentary change visible in the fundus pre-operatively and/or post-mortem. Within 1 mm of the PP termination of BM there was PE atrophy (9/10 eyes), choroidal atrophy (9/9), thickened outer BM (8/9), and CNV (2/10). Deposits in inner BM (7/10) consisted of heterogenous debris in the inner collagenous zone and thickened PE-associated basement membrane (preliminary data). Within 0.2 mm of the PP termination of the photoreceptor layer, rod density declined sharply, leaving mostly cones. Conclusions: PPCRA is associated with PE atrophy, inner BM changes, and marked loss of rods. AMD also has major BM changes and risk for CNV. The sublaminar location of the most prominent BM changes in PPCRA may differ from that of AMD.
  • Author List

  • Curcio CA; Saunders PL; Younger PW; Medeiros NE; Millican CL
  • Volume

  • 37
  • Issue

  • 3