PMID- 28365912
OWN - NLM
STAT- MEDLINE
DCOM- 20171120
LR  - 20240109
IS  - 1435-702X (Electronic)
IS  - 0721-832X (Print)
IS  - 0721-832X (Linking)
VI  - 255
IP  - 7
DP  - 2017 Jul
TI  - Multimodal analysis of the Preferred Retinal Location and the Transition Zone in 
      patients with Stargardt Disease.
PG  - 1307-1317
LID - 10.1007/s00417-017-3637-6 [doi]
AB  - PURPOSE: The purpose of our study was to investigate morpho-functional features 
      of the preferred retinal location (PRL) and the transition zone (TZ) in a series 
      of patients with recessive Stargardt disease (STGD1). METHODS: Fifty-two STGD1 
      patients with at least one ABCA4 mutation, atrophy of the central macula (MA) and 
      an eccentric PRL were recruited for the study. Microperimetry, fundus 
      autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT) 
      were performed. The location and stability of the PRL along with the associated 
      FAF pattern and visual sensitivities were determined and compared to the 
      underlying retinal structure. RESULTS: The mean visual sensitivity of the PRLs 
      for the 52 eyes was 10.76 +/- 3.70 dB. For the majority of eyes, PRLs were 
      associated with intact ellipsoid zone (EZ) bands and qualitatively normal FAF 
      patterns. In 17 eyes (32.7%) the eccentric PRL was located at the edge of the MA. 
      In 35 eyes (67.3%) it was located at varying distances from the border of the MA 
      with a TZ between the PRL and the MA. The TZ was associated with decreased 
      sensitivity values (5.92 +/- 4.69 dB) compared to PRLs (p<0.05), with 
      absence/disruption of the EZ band and abnormal FAF patterns (hyper or 
      hypo-autofluorescence). CONCLUSIONS: In STGD1 eccentric PRLs are located away 
      from the border of MA and associated with intact EZ bands and normal FAF. The TZ 
      is characterized by structural and functional abnormalities. The results of 
      multimodal imaging of the PRL and TZ suggest a possible sequence of retinal and 
      functional changes with disease progression that may help in the planning of 
      future therapies; RPE dysfunction appears to be the primary event leading to 
      photoreceptor degeneration and then to RPE loss.
FAU - Verdina, Tommaso
AU  - Verdina T
AUID- ORCID: 0000-0001-7877-4485
AD  - Institute of Ophthalmology, University of Modena and Reggio Emilia, Via del Pozzo 
      71, 41100, Modena, Italy. tommaso.verdina@gmail.com.
FAU - Greenstein, Vivienne C
AU  - Greenstein VC
AD  - Department of Ophthalmology, Columbia University, New York, NY, USA.
FAU - Sodi, Andrea
AU  - Sodi A
AD  - Department of Translational Surgery and Medicine, Eye Clinic, University of 
      Florence, Largo Brambilla 3, Florence, 50134, Italy.
FAU - Tsang, Stephen H
AU  - Tsang SH
AD  - Department of Ophthalmology, Columbia University, New York, NY, USA.
AD  - Barbara & Donald Jonas Stem Cell & Regenerative Medicine Laboratory, and Bernard 
      & Shirlee Brown Glaucoma Laboratory, Departments of Ophthalmology, Pathology & 
      Cell Biology, Institute of Human Nutrition, College of Physicians and Surgeons, 
      Columbia University, New York, NY, 10032, USA.
FAU - Burke, Tomas R
AU  - Burke TR
AD  - Department of Ophthalmology, Columbia University, New York, NY, USA.
FAU - Passerini, Ilaria
AU  - Passerini I
AD  - Department of Genetic Diagnostics, Azienda Ospedaliero Universitaria Careggi, 
      Florence, Italy.
FAU - Allikmets, Rando
AU  - Allikmets R
AD  - Department of Ophthalmology, Columbia University, New York, NY, USA.
AD  - Barbara & Donald Jonas Stem Cell & Regenerative Medicine Laboratory, and Bernard 
      & Shirlee Brown Glaucoma Laboratory, Departments of Ophthalmology, Pathology & 
      Cell Biology, Institute of Human Nutrition, College of Physicians and Surgeons, 
      Columbia University, New York, NY, 10032, USA.
FAU - Virgili, Gianni
AU  - Virgili G
AD  - Department of Translational Surgery and Medicine, Eye Clinic, University of 
      Florence, Largo Brambilla 3, Florence, 50134, Italy.
FAU - Cavallini, Gian Maria
AU  - Cavallini GM
AD  - Institute of Ophthalmology, University of Modena and Reggio Emilia, Via del Pozzo 
      71, 41100, Modena, Italy.
FAU - Rizzo, Stanislao
AU  - Rizzo S
AD  - Department of Translational Surgery and Medicine, Eye Clinic, University of 
      Florence, Largo Brambilla 3, Florence, 50134, Italy.
LA  - eng
GR  - R21 AG050437/AG/NIA NIH HHS/United States
GR  - R01 EY018213/EY/NEI NIH HHS/United States
GR  - R01 EY009076/EY/NEI NIH HHS/United States
GR  - R01 EY026682/EY/NEI NIH HHS/United States
GR  - R01 EY024698/EY/NEI NIH HHS/United States
PT  - Journal Article
DEP - 20170402
PL  - Germany
TA  - Graefes Arch Clin Exp Ophthalmol
JT  - Graefe's archive for clinical and experimental ophthalmology = Albrecht von 
      Graefes Archiv fur klinische und experimentelle Ophthalmologie
JID - 8205248
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Fluorescein Angiography/methods
MH  - Fundus Oculi
MH  - Humans
MH  - Macula Lutea/*pathology
MH  - Macular Degeneration/*congenital/diagnosis/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Multimodal Imaging/*methods
MH  - Ophthalmoscopy/methods
MH  - Reproducibility of Results
MH  - Retinal Pigment Epithelium/*pathology
MH  - Retrospective Studies
MH  - Stargardt Disease
MH  - Tomography, Optical Coherence/methods
MH  - Visual Acuity
MH  - Visual Fields
MH  - Young Adult
PMC - PMC5500688
MID - NIHMS864985
OTO - NOTNLM
OT  - Eccentric fixation
OT  - Fundus autofluorescence
OT  - Fundus flavimaculatus
OT  - Microperimetry
OT  - Preferred retinal location
OT  - SD-OCT
OT  - Stargardt disease
COIS- Conflict of Interest: All authors certify that they have no affiliations with or 
      involvement in any organization or entity with any financial interest (such as 
      honoraria; educational grants; participation in speakers' bureaus; membership, 
      employment, consultancies, stock ownership, or other equity interest; and expert 
      testimony or patent-licensing arrangements), or non-financial interest (such as 
      personal or professional relationships, affiliations, knowledge or beliefs) in 
      the subject matter or materials discussed in this manuscript.
EDAT- 2017/04/04 06:00
MHDA- 2017/11/29 06:00
PMCR- 2018/07/01
CRDT- 2017/04/03 06:00
PHST- 2016/09/01 00:00 [received]
PHST- 2017/03/13 00:00 [accepted]
PHST- 2016/12/29 00:00 [revised]
PHST- 2017/04/04 06:00 [pubmed]
PHST- 2017/11/29 06:00 [medline]
PHST- 2017/04/03 06:00 [entrez]
PHST- 2018/07/01 00:00 [pmc-release]
AID - 10.1007/s00417-017-3637-6 [pii]
AID - 10.1007/s00417-017-3637-6 [doi]
PST - ppublish
SO  - Graefes Arch Clin Exp Ophthalmol. 2017 Jul;255(7):1307-1317. doi: 
      10.1007/s00417-017-3637-6. Epub 2017 Apr 2.