title: Rates of decline in regions of the visual field defined by frequency-domain optical coherence tomography in patients with RPGR-mediated X-linked retinitis pigmentosa.
abstract: OBJECTIVE
  To determine whether annual decline in visual field sensitivity is greater in the transition zone at the edge of the frequency-domain optical coherence tomography (fdOCT) inner segment ellipsoid zone (EZ) than at other locations in the visual field.
  
  
  METHODS
  Prospective, longitudinal, observational study.
  
  
  METHODS
  Forty-four patients with X-linked retinitis pigmentosa (XLRP) resulting from a mutation in the RPGR gene.
  
  
  METHODS
  Static perimetric fields (Humphrey 30-2; Carl Zeiss Meditec, Dublin, CA) were obtained annually for 4 years. Beginning with year 2, fdOCT scans were obtained annually with a Heidelberg Spectralis HRA + OCT (Heidelberg Engineering, Heidelberg, Germany).
  
  
  METHODS
  The rate of visual field decline at locations near the edge of the EZ compared with the rates for the macula and in the mid periphery.
  
  
  RESULTS
  Sensitivity just inside and outside the edge of the EZ declined at rates of 0.84 and 0.92 dB/year, respectively. By comparison, average sensitivity in the macula and mid periphery declined by 0.38 and 0.61 dB/year, respectively.
  
  
  CONCLUSIONS
  The edge of the EZ in each patient with XLRP indicates a transition zone between relatively healthy and relatively degenerate retina. The annual loss of sensitivity in the transition zone is more rapid than it is elsewhere in the retina.
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas; Department of Ophthalmology, The University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address: dbirch@retinafoundation.org.
authors.forename: David G
authors.initials: DG
authors.lastname: Birch
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas.
authors.forename: Kirsten G
authors.initials: KG
authors.lastname: Locke
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas; Department of Ophthalmology, The University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Joost
authors.initials: J
authors.lastname: Felius
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas.
authors.forename: Martin
authors.initials: M
authors.lastname: Klein
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas; Department of Ophthalmology, The University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Dianna K H
authors.initials: DK
authors.lastname: Wheaton
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas; Department of Ophthalmology, The University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Dennis R
authors.initials: DR
authors.lastname: Hoffman
authors.affiliation: Departments of Psychology and Ophthalmology, Columbia University, New York, New York.
authors.forename: Donald C
authors.initials: DC
authors.lastname: Hood
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005136:Eye Proteins / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D008297:Male; D009154:Mutation; D011446:Prospective Studies; D012160:Retina / Q000503:physiopathology*; D012174:Retinitis Pigmentosa / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D012680:Sensitivity and Specificity; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000503:physiopathology*; D014792:Visual Acuity / Q000502:physiology; D058609:Visual Field Tests; D014794:Visual Fields / Q000502:physiology*; D055815:Young Adult
chemical_list: D005136:Eye Proteins; C099929:RPGR protein, human