Fundus autofluorescence in type 2 idiopathic macular telangiectasia: correlation with optical coherence tomography and microperimetry.
OBJECTIVE
To use multiple imaging methods to investigate patients with type 2 idiopathic macular telangiectasia (IMT) at different disease severity stages so as to characterize and categorize disease progression through the full spectrum of disease phenotypes.


METHODS
Observational case series.


METHODS
Twelve patients with type 2 IMT (22 eyes) examined with fundus photography, angiography, optical coherence tomography imaging, fundus autofluorescence (FAF), and microperimetry testing in an institutional setting.


RESULTS
Eyes examined by multiple imaging methods were classified into 5 proposed categories (0 through 4): category 0 (fellow) eyes had normal results on all imaging methods. Category 1 eyes had increased foveal autofluorescence on FAF imaging as the only imaging abnormality. Category 2 eyes had increased foveal autofluorescence together with funduscopic and angiographic features typical of type 2 IMT. Category 3 eyes had additional evidence of foveal atrophy on optical coherence tomography, and category 4 eyes had all the above features plus clinically evident pigment clumping. FAF signal increased in intensity in the foveal region from category 0 through category 3, whereas category 4 eyes demonstrated a mixed pattern of increased and decreased FAF signal.


CONCLUSIONS
The findings here outline a sequence of progressive changes seen with multiple imaging methods in advancing stages of disease. Increase in foveal autofluorescence is an early anatomic change in type 2 IMT that may precede typical clinical and angiographic changes. Loss of macular pigment density in the fovea and a changing composition of fluorophores in the retinal pigment epithelium may underlie these changes on FAF in the fundus.
Wong
Wai T
WT
Office of the Scientific Director, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA.
Forooghian
Farzin
F
Majumdar
Zigurts
Z
Bonner
Robert F
RF
Cunningham
Denise
D
Chew
Emily Y
EY
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004396:Coloring Agents; D003430:Cross-Sectional Studies; D003952:Diagnostic Imaging / Q000379:methods*; D003941:Diagnostic Techniques, Ophthalmological; D018450:Disease Progression; D005260:Female; D005451:Fluorescein Angiography; D005453:Fluorescence; D005654:Fundus Oculi; D006801:Humans; D007208:Indocyanine Green; D008297:Male; D008875:Middle Aged; D010781:Photography; D012164:Retinal Diseases / Q000175:diagnosis* / Q000503:physiopathology; D012171:Retinal Vessels / Q000473:pathology*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013684:Telangiectasis / Q000175:diagnosis* / Q000503:physiopathology; D041623:Tomography, Optical Coherence; D058609:Visual Field Tests
D004396:Coloring Agents; D007208:Indocyanine Green