Characterization of visual function, interocular variability and progression using static perimetry-derived metrics in RPGR-associated retinopathy

James J.L. Tee, Yesa Yang, Angelos Kalitzeos, Andrew Webster, James Bainbridge, Richard Weleber, Michel Michaelides

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

PURPOSE. To characterize bilateral visual function, interocular variability and progression by using static perimetry-derived volumetric and pointwise metrics in subjects with retinitis pigmentosa associated with mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. METHODS. This was a prospective longitudinal observational study of 47 genetically confirmed subjects. Visual function was assessed with ETDRS and Pelli-Robson charts; and Octopus 900 static perimetry using a customized, radially oriented 185-point grid. Three-dimensional hillof-vision topographic models were produced and interrogated with the Visual Field Modeling and Analysis software to obtain three volumetric metrics: VTotal, V30, and V5. These were analyzed together with Octopus mean sensitivity values. Interocular differences were assessed with the Bland-Altman method. Metric-specific exponential decline rates were calculated. RESULTS. Baseline symmetry was demonstrated by relative interocular difference values of 1% for VTotaland 8% with V30. Degree of symmetry varied between subjects and was quantified with the subject percentage interocular difference (SPID). SPID was 16% for VTotaland 17% for V30. Interocular symmetry in progression was greatest when quantified by VTotaland V30, with 73% and 64% of subjects possessing interocular rate differences smaller in magnitude than respective annual progression rates. Functional decline was evident with increasing age. An overall annual exponential decline of 6% was evident with both VTotaland V30. CONCLUSIONS. In general, good interocular symmetry exists; however, there was both variation between subjects and with the use of various metrics. Our findings will guide patient selection and design of RPGR treatment trials, and provide clinicians with specific prognostic information to offer patients affected by this condition.

Original languageEnglish (US)
Pages (from-to)2422-2436
Number of pages15
JournalInvestigative Ophthalmology and Visual Science
Volume59
Issue number6
DOIs
StatePublished - May 1 2018

Fingerprint

Visual Field Tests
Retinitis Pigmentosa
GTP Phosphohydrolases
Octopodiformes
Regulator Genes
Visual Fields
Patient Selection
Observational Studies
Longitudinal Studies
Software
Mutation
Therapeutics

Keywords

  • Genetic diseases
  • Retinitis pigmentosa
  • RPGR
  • Static perimetry
  • Visual field

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this

Characterization of visual function, interocular variability and progression using static perimetry-derived metrics in RPGR-associated retinopathy. / Tee, James J.L.; Yang, Yesa; Kalitzeos, Angelos; Webster, Andrew; Bainbridge, James; Weleber, Richard; Michaelides, Michel.

In: Investigative Ophthalmology and Visual Science, Vol. 59, No. 6, 01.05.2018, p. 2422-2436.

Research output: Contribution to journalArticle

Tee, James J.L. ; Yang, Yesa ; Kalitzeos, Angelos ; Webster, Andrew ; Bainbridge, James ; Weleber, Richard ; Michaelides, Michel. / Characterization of visual function, interocular variability and progression using static perimetry-derived metrics in RPGR-associated retinopathy. In: Investigative Ophthalmology and Visual Science. 2018 ; Vol. 59, No. 6. pp. 2422-2436.
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abstract = "PURPOSE. To characterize bilateral visual function, interocular variability and progression by using static perimetry-derived volumetric and pointwise metrics in subjects with retinitis pigmentosa associated with mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. METHODS. This was a prospective longitudinal observational study of 47 genetically confirmed subjects. Visual function was assessed with ETDRS and Pelli-Robson charts; and Octopus 900 static perimetry using a customized, radially oriented 185-point grid. Three-dimensional hillof-vision topographic models were produced and interrogated with the Visual Field Modeling and Analysis software to obtain three volumetric metrics: VTotal, V30, and V5. These were analyzed together with Octopus mean sensitivity values. Interocular differences were assessed with the Bland-Altman method. Metric-specific exponential decline rates were calculated. RESULTS. Baseline symmetry was demonstrated by relative interocular difference values of 1{\%} for VTotaland 8{\%} with V30. Degree of symmetry varied between subjects and was quantified with the subject percentage interocular difference (SPID). SPID was 16{\%} for VTotaland 17{\%} for V30. Interocular symmetry in progression was greatest when quantified by VTotaland V30, with 73{\%} and 64{\%} of subjects possessing interocular rate differences smaller in magnitude than respective annual progression rates. Functional decline was evident with increasing age. An overall annual exponential decline of 6{\%} was evident with both VTotaland V30. CONCLUSIONS. In general, good interocular symmetry exists; however, there was both variation between subjects and with the use of various metrics. Our findings will guide patient selection and design of RPGR treatment trials, and provide clinicians with specific prognostic information to offer patients affected by this condition.",
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AU - Webster, Andrew

AU - Bainbridge, James

AU - Weleber, Richard

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N2 - PURPOSE. To characterize bilateral visual function, interocular variability and progression by using static perimetry-derived volumetric and pointwise metrics in subjects with retinitis pigmentosa associated with mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. METHODS. This was a prospective longitudinal observational study of 47 genetically confirmed subjects. Visual function was assessed with ETDRS and Pelli-Robson charts; and Octopus 900 static perimetry using a customized, radially oriented 185-point grid. Three-dimensional hillof-vision topographic models were produced and interrogated with the Visual Field Modeling and Analysis software to obtain three volumetric metrics: VTotal, V30, and V5. These were analyzed together with Octopus mean sensitivity values. Interocular differences were assessed with the Bland-Altman method. Metric-specific exponential decline rates were calculated. RESULTS. Baseline symmetry was demonstrated by relative interocular difference values of 1% for VTotaland 8% with V30. Degree of symmetry varied between subjects and was quantified with the subject percentage interocular difference (SPID). SPID was 16% for VTotaland 17% for V30. Interocular symmetry in progression was greatest when quantified by VTotaland V30, with 73% and 64% of subjects possessing interocular rate differences smaller in magnitude than respective annual progression rates. Functional decline was evident with increasing age. An overall annual exponential decline of 6% was evident with both VTotaland V30. CONCLUSIONS. In general, good interocular symmetry exists; however, there was both variation between subjects and with the use of various metrics. Our findings will guide patient selection and design of RPGR treatment trials, and provide clinicians with specific prognostic information to offer patients affected by this condition.

AB - PURPOSE. To characterize bilateral visual function, interocular variability and progression by using static perimetry-derived volumetric and pointwise metrics in subjects with retinitis pigmentosa associated with mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. METHODS. This was a prospective longitudinal observational study of 47 genetically confirmed subjects. Visual function was assessed with ETDRS and Pelli-Robson charts; and Octopus 900 static perimetry using a customized, radially oriented 185-point grid. Three-dimensional hillof-vision topographic models were produced and interrogated with the Visual Field Modeling and Analysis software to obtain three volumetric metrics: VTotal, V30, and V5. These were analyzed together with Octopus mean sensitivity values. Interocular differences were assessed with the Bland-Altman method. Metric-specific exponential decline rates were calculated. RESULTS. Baseline symmetry was demonstrated by relative interocular difference values of 1% for VTotaland 8% with V30. Degree of symmetry varied between subjects and was quantified with the subject percentage interocular difference (SPID). SPID was 16% for VTotaland 17% for V30. Interocular symmetry in progression was greatest when quantified by VTotaland V30, with 73% and 64% of subjects possessing interocular rate differences smaller in magnitude than respective annual progression rates. Functional decline was evident with increasing age. An overall annual exponential decline of 6% was evident with both VTotaland V30. CONCLUSIONS. In general, good interocular symmetry exists; however, there was both variation between subjects and with the use of various metrics. Our findings will guide patient selection and design of RPGR treatment trials, and provide clinicians with specific prognostic information to offer patients affected by this condition.

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