title: Structure/Psychophysical Relationships in X-Linked Retinoschisis.
abstract: OBJECTIVE
  To compare structural properties from spectral-domain optical coherence tomography (SDOCT) and psychophysical measures from a subset of patients enrolled in a larger multicenter natural history study of X-linked retinoschisis (XLRS).
  
  
  METHODS
  A subset of males (n = 24) participating in a larger natural history study of XLRS underwent high-resolution SDOCT. Total retina (TR) thickness and outer segment (OS) thickness were measured manually. Shape discrimination hyperacuity (SDH) and contour integration perimetry (CIP) were performed on an iPad with the myVisionTrack application. Sensitivity was measured with fundus-guided perimetry (4-2 threshold testing strategy; 10-2 grid, spot size 3, 68 points). Correlation was determined with Pearson's r correlation. Values are presented as the mean ± SD.
  
  
  RESULTS
  Mean macular OS thickness was less in XLRS patients (17.2 ± 8.1 μm) than in controls (37.1 ± 5.7 μm; P < 0.0001) but mean TR thickness was comparable (P = 0.5884). For patients, total sensitivity was lower (13.2 ± 6.6 dB) than for controls (24.2 ± 2.4 dB; P = 0.0008) and had a strong correlation with photoreceptor OS (R(2) = 0.55, P = 0.0001) and a weak correlation with TR thickness (R(2) = 0.22, P = 0.0158). The XLRS subjects had a logMAR best corrected visual acuity (BCVA) of 0.5 ± 0.3 that was associated with OS (R(2) = 0.79, P < 0.0001) but not TR thickness (R(2) = 0.01, P = 0.6166). Shape DH and CIP inner ring correlated with OS (R(2) = 0.33, P = 0.0085 and R(2) = 0.47, P = 0.0001, respectively) but not TR thickness (R(2) = 0.0004, P = 0.93; R(2) = 0.0043, P = 0.75, respectively).
  
  
  CONCLUSIONS
  When considered from a single visit, OS thickness within the macula is more closely associated with macular function than TR thickness within the macula in patients with XLRS.
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas, United States.
authors.forename: Lea D
authors.initials: LD
authors.lastname: Bennett
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas, United States 2Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, United States.
authors.forename: Yi-Zhong
authors.initials: YZ
authors.lastname: Wang
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas, United States.
authors.forename: Martin
authors.initials: M
authors.lastname: Klein
authors.affiliation: Oregon Health & Science University, Casey Eye Institute, Portland, Oregon, United States.
authors.forename: Mark E
authors.initials: ME
authors.lastname: Pennesi
authors.affiliation: University of Michigan, Kellogg Eye Center, Ann Arbor, Michigan, United States.
authors.forename: Thiran
authors.initials: T
authors.lastname: Jayasundera
authors.affiliation: Retina Foundation of the Southwest, Dallas, Texas, United States 2Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, United States.
authors.forename: David G
authors.initials: DG
authors.lastname: Birch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005136:Eye Proteins / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D011601:Psychophysics; D012160:Retina / Q000503:physiopathology*; D055214:Retinal Photoreceptor Cell Outer Segment / Q000473:pathology*; D041441:Retinoschisis / Q000235:genetics / Q000503:physiopathology*; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology*; D058609:Visual Field Tests; D014794:Visual Fields / Q000502:physiology
chemical_list: D005136:Eye Proteins; C108726:RS1 protein, human
references.citation: Surv Ophthalmol. 2008 Jan-Feb;53(1):16-40
references.pmid: 18191655
references.citation: Can J Ophthalmol. 1998 Apr;33(3):149-58
references.pmid: 9606571
references.citation: Ophthalmology. 2005 Mar;112(3):495-501
references.pmid: 15745780
references.citation: Invest Ophthalmol Vis Sci. 2011 Nov;52(12):9250-6
references.pmid: 22039241
references.citation: Retina. 2011 Jun;31(6):1186-92
references.pmid: 21386765
references.citation: Nat Genet. 1997 Oct;17(2):164-70
references.pmid: 9326935
references.citation: Retina. 2006 Sep;26(7):741-5
references.pmid: 16963845
references.citation: Transl Vis Sci Technol. 2013 Nov;2(7):5
references.pmid: 24349883
references.citation: Br J Ophthalmol. 1995 Jul;79(7):653-7
references.pmid: 7662629
references.citation: Invest Ophthalmol Vis Sci. 2013 Aug;54(8):5497-505
references.pmid: 23860761
references.citation: Retina. 2011 Feb;31(2):312-5
references.pmid: 20966823
references.citation: Ophthalmology. 2014 Mar;121(3):802-3.e3
references.pmid: 24309554
references.citation: Fortschr Ophthalmol. 1990;87(3):264-8
references.pmid: 2376375
references.citation: Optom Vis Sci. 2001 Jun;78(6):447-54
references.pmid: 11444636
references.citation: Invest Ophthalmol Vis Sci. 2014 May;55(5):3029-36
references.pmid: 24713485
references.citation: Surv Ophthalmol. 2004 Mar-Apr;49(2):214-30
references.pmid: 14998693
references.citation: Am J Ophthalmol. 2009 Jan;147(1):111-115.e1
references.pmid: 18834580
references.citation: Arch Ophthalmol. 1987 Apr;105(4):513-6
references.pmid: 3566604
references.citation: Mol Vis. 2008;14:2321-32
references.pmid: 19093009
references.citation: Acta Ophthalmol Scand. 2004 Apr;82(2):218-23
references.pmid: 15043546
references.citation: Vision Res. 2001 Dec;41(28):3931-42
references.pmid: 11738458
references.citation: Ophthalmic Genet. 2010 Sep;31(3):114-25
references.pmid: 20569020
references.citation: Transl Vis Sci Technol. 2014 Oct 3;3(5):5
references.pmid: 25346871
references.citation: Arch Ophthalmol. 2000 Aug;118(8):1098-104
references.pmid: 10922205