title: MULTIMODAL IMAGING OF POSTERIOR POLAR ANNULAR CHOROIDAL DYSTROPHY.
abstract: BACKGROUND
  Posterior polar annular choroidal dystrophy (PPACD) is a rare disease. Patients with PPACD show loss of retinal pigment epithelium and choriocapillaries surrounding the vascular arcades and optic nerve.
  
  
  METHODS
  Two patients with PPACD were evaluated with multimodal imaging, including fundus autofluorescence (FAF) and adaptive optics (AO).
  
  
  METHODS
  One patient (32 year old, one eye) with PPACD was followed up for 3 years. Best-corrected visual acuity (BCVA) was stable at 20/40, whereas a slight enlargement of paravascular atrophy of pigment epithelium was observed at fundus autofluorescence (FAF). Adaptive optics obtained at last examination showed reduced density of foveal cone photoreceptors. The second patient (30 year old, two eyes) with PPACD showed bilateral normal BCVA, associated with reduction in the density of foveal cone photoreceptors.
  
  
  CONCLUSIONS
  At FAF, longitudinal follow-up of PPACD showed progression of the paravascular atrophy of the pigment epithelium. Foveal cone photoreceptors can be reduced even in the presence of preserved visual acuity.
authors: Department of Ophthalmology, Grenoble Alpes University, University Hospital of Grenoble, Grenoble, France.
authors: Raimondo
authors: R
authors: Forte
authors: Florent
authors: F
authors: Aptel
authors: Audrey
authors: A
authors: Feldmann
authors: Christophe
authors: C
authors: Chiquet
mesh_terms: D000328:Adult; D002829:Choroid / Q000473:pathology*; D015862:Choroid Diseases / Q000175:diagnosis*; D005451:Fluorescein Angiography / Q000379:methods*; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D064847:Multimodal Imaging; D035583:Rare Diseases; D017949:Retinal Cone Photoreceptor Cells / Q000473:pathology*; D055213:Retinal Pigment Epithelium / Q000473:pathology; D041623:Tomography, Optical Coherence / Q000379:methods*; D014792:Visual Acuity