PMID- 25587056
OWN - NLM
STAT- MEDLINE
DCOM- 20150324
LR  - 20220330
IS  - 1552-5783 (Electronic)
IS  - 0146-0404 (Print)
IS  - 0146-0404 (Linking)
VI  - 56
IP  - 2
DP  - 2015 Jan 13
TI  - Adaptive optics microperimetry and OCT images show preserved function and 
      recovery of cone visibility in macular telangiectasia type 2 retinal lesions.
PG  - 778-86
LID - 10.1167/iovs.14-15576 [doi]
AB  - PURPOSE: To evaluate visual function and disease progression in the retinal 
      structural abnormalities of three patients from two unrelated families with 
      macular telangiectasia (MacTel) type 2. METHODS: Adaptive optics scanning laser 
      ophthalmoscopy (AOSLO) and AOSLO microperimetry (AOMP) were used to evaluate the 
      structure and function of macular cones in three eyes with MacTel type 2. Cone 
      spacing was estimated using histogram analysis of intercone distances, and 
      registered spectral-domain optical coherence tomography (SD-OCT) scans were used 
      to evaluate retinal anatomy. AOMP was used to assess visual sensitivity in and 
      around areas of apparent cone loss. RESULTS: Although overall lesion surface area 
      increased, some initially affected regions subsequently showed clear, contiguous, 
      and normally spaced cone mosaics with recovered photoreceptor inner/outer segment 
      (IS/OS) reflectivity (two of two eyes). The AOMP test sites fell within three 
      categories: normal-appearing cones (N), dimly reflecting cones (D), and RPE cell 
      mosaics (R). At N sites, AOMP threshold values (arbitrary units [au]) increased 
      with increasing eccentricity (slope = 0.054 au/degree, r(2) = 0.77). The N 
      thresholds ranged from 0.04 to 0.27 au, D thresholds from 0.04 to 0.33 au, and R 
      thresholds from 0.14 to 1.00 au. There was measurable visual sensitivity 
      everywhere except areas without intact external limiting membrane (ELM) and with 
      diffuse scattering in the IS/OS and posterior tips of the outer segments (PTOS) 
      regions on OCT. CONCLUSIONS: Visual sensitivity and recovery of cone visibility 
      in areas of apparent focal cone loss suggests that MacTel type 2 lesions with a 
      preserved ELM may contain functioning cones with abnormal scattering and/or 
      waveguiding characteristics. (ClinicalTrials.gov number, NCT00254605.).
CI  - Copyright 2015 The Association for Research in Vision and Ophthalmology, Inc.
FAU - Wang, Qinyun
AU  - Wang Q
AD  - Duke University School of Medicine, Durham, North Carolina, United States 
      Department of Ophthalmology, University of California, San Francisco, San 
      Francisco, California, United States.
FAU - Tuten, William S
AU  - Tuten WS
AD  - School of Optometry and Vision Science Graduate Group, University of California, 
      Berkeley, Berkeley, California, United States.
FAU - Lujan, Brandon J
AU  - Lujan BJ
AD  - School of Optometry and Vision Science Graduate Group, University of California, 
      Berkeley, Berkeley, California, United States West Coast Retina Medical Group, 
      San Francisco, California, United States.
FAU - Holland, Jennifer
AU  - Holland J
AD  - Department of Ophthalmology, University of California, San Francisco, San 
      Francisco, California, United States.
FAU - Bernstein, Paul S
AU  - Bernstein PS
AD  - Department of Ophthalmology, University of Utah Moran Eye Center, Salt Lake City, 
      Utah, United States.
FAU - Schwartz, Steven D
AU  - Schwartz SD
AD  - Jules Stein Eye Institute, University of California, Los Angeles, Los Angeles, 
      California, United States.
FAU - Duncan, Jacque L
AU  - Duncan JL
AD  - Department of Ophthalmology, University of California, San Francisco, San 
      Francisco, California, United States.
FAU - Roorda, Austin
AU  - Roorda A
AD  - School of Optometry and Vision Science Graduate Group, University of California, 
      Berkeley, Berkeley, California, United States.
LA  - eng
SI  - ClinicalTrials.gov/NCT00254605
GR  - EY014375/EY/NEI NIH HHS/United States
GR  - EY022412/EY/NEI NIH HHS/United States
GR  - L30 EY021930/EY/NEI NIH HHS/United States
GR  - R01 EY023591/EY/NEI NIH HHS/United States
GR  - EY002162/EY/NEI NIH HHS/United States
GR  - EY023591/EY/NEI NIH HHS/United States
GR  - K23 EY022412/EY/NEI NIH HHS/United States
GR  - R01 EY014375/EY/NEI NIH HHS/United States
GR  - P30 EY002162/EY/NEI NIH HHS/United States
PT  - Journal Article
PT  - Randomized Controlled Trial
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20150113
PL  - United States
TA  - Invest Ophthalmol Vis Sci
JT  - Investigative ophthalmology & visual science
JID - 7703701
RN  - Osler-rendu-weber syndrome 2
SB  - IM
MH  - Adult
MH  - Female
MH  - Fluorescein Angiography
MH  - Follow-Up Studies
MH  - Fundus Oculi
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Ophthalmoscopy/methods
MH  - *Recovery of Function
MH  - Retinal Cone Photoreceptor Cells/*physiology
MH  - Telangiectasia, Hereditary Hemorrhagic/*diagnosis/physiopathology
MH  - Tomography, Optical Coherence/*methods
MH  - Visual Acuity
MH  - Visual Field Tests/*methods
MH  - Visual Fields/*physiology
PMC - PMC4315435
OTO - NOTNLM
OT  - AOMP
OT  - AOSLO
OT  - MacTel type 2
OT  - cone photoreceptors
EDAT- 2015/01/15 06:00
MHDA- 2015/03/25 06:00
PMCR- 2015/08/01
CRDT- 2015/01/15 06:00
PHST- 2015/01/15 06:00 [entrez]
PHST- 2015/01/15 06:00 [pubmed]
PHST- 2015/03/25 06:00 [medline]
PHST- 2015/08/01 00:00 [pmc-release]
AID - iovs.14-15576 [pii]
AID - 10.1167/iovs.14-15576 [doi]
PST - epublish
SO  - Invest Ophthalmol Vis Sci. 2015 Jan 13;56(2):778-86. doi: 10.1167/iovs.14-15576.