PMID- 38484106
OWN - NLM
STAT- Publisher
LR  - 20240411
IS  - 1539-2864 (Electronic)
IS  - 0275-004X (Linking)
DP  - 2024 Mar 13
TI  - Longitudinal Adaptive Optics Scanning Laser Ophthalmoscopy Reveals Regional 
      Variation in Cone and Rod Photoreceptor Loss in Stargardt Disease.
LID - 10.1097/IAE.0000000000004104 [doi]
AB  - PURPOSE: To investigate the temporal sequence of changes in the photoreceptor 
      cell mosaic in patients with Stargardt disease type 1 (STGD1), using adaptive 
      optics scanning laser ophthalmoscopy (AOSLO). METHODS: Two brothers with 
      genetically confirmed STGD1 underwent comprehensive eye exams, spectral-domain 
      optical coherence tomography (SD-OCT), fundus auto fluorescence (FAF) and AOSLO 
      imaging 3 times over the course of 28 months. Confocal images of the cones and 
      rods were obtained from the central fovea to 10 degrees inferiorly. 
      Photoreceptors were counted in sampling windows at 100 microm intervals of 200 microm x 
      200 microm for cones and 50 microm x 50 microm for rods, using custom cell marking software 
      with manual correction. Photoreceptor density and spacing were measured and 
      compared across imaging sessions using one-way ANOVA. RESULTS: AOSLO revealed the 
      younger brother had a 30% decline in foveal cone density after 8 months, followed 
      by complete loss of foveal cones at 28 months; the older brother had no 
      detectable foveal cones at baseline. In the peripheral macula, cone and rod 
      spacings were greater than normal in both patients. The ratio of the cone spacing 
      to rod spacing was greater than normal across all eccentricities, with a greater 
      divergence closer to the foveal center. CONCLUSIONS: Cone cell loss may be an 
      early pathogenetic step in Stargardt disease. AOSLO provides the capability to 
      track individual photoreceptor changes longitudinally in Stargardt disease. 
      SUMMARY STATEMENT: The pathogenetic mechanism of Stargardt disease remains poorly 
      understood. We used high resolution AOSLO to track the progression of the disease 
      and found cone cell loss may be an early pathogenetic step in Stargardt disease.
FAU - Song, Hongxin
AU  - Song H
AD  - Beijing Tongren Eye Center, Beijing Institute of Ophthalmology, Beijing Tongren 
      Hospital, Capital Medical University, Beijing Key Laboratory of Ophthalmology and 
      visual Sciences, National Engineering Research Center for Ophthalmology. Beijing, 
      China.
FAU - Hang, Hui
AU  - Hang H
AUID- ORCID: 0000-0002-7652-0032
AD  - Department of Ophthalmology, Jiangsu Province Hospital and Nanjing Medical 
      University First Affiliated Hospital. Nanjing, China.
FAU - Li, Kaiwen
AU  - Li K
AD  - Advanced Ophthalmology Laboratory (AOL), Robotrak Technologies, Nanjing, China.
FAU - Rossi, Ethan A
AU  - Rossi EA
AD  - Department of Ophthalmology, University of Pittsburgh, Pittsburgh, Pennsylvania, 
      USA.
FAU - Zhang, Jie
AU  - Zhang J
AD  - Advanced Ophthalmology Laboratory (AOL), Robotrak Technologies, Nanjing, China.
LA  - eng
GR  - P30 EY001319/EY/NEI NIH HHS/United States
GR  - R01 EY014375/EY/NEI NIH HHS/United States
GR  - R01 EY021786/EY/NEI NIH HHS/United States
PT  - Journal Article
DEP - 20240313
PL  - United States
TA  - Retina
JT  - Retina (Philadelphia, Pa.)
JID - 8309919
SB  - IM
EDAT- 2024/03/14 18:42
MHDA- 2024/03/14 18:42
CRDT- 2024/03/14 14:22
PHST- 2024/03/14 18:42 [medline]
PHST- 2024/03/14 18:42 [pubmed]
PHST- 2024/03/14 14:22 [entrez]
AID - 00006982-990000000-00630 [pii]
AID - 10.1097/IAE.0000000000004104 [doi]
PST - aheadofprint
SO  - Retina. 2024 Mar 13. doi: 10.1097/IAE.0000000000004104.