Macular pigment and visual acuity in Stargardt macular dystrophy.
OBJECTIVE
To test the hypothesis that macular pigment reflects foveal cone function and possibly the presence of foveal cones in recessive Stargardt macular dystrophy.


METHODS
Sixteen patients (32 eyes) diagnosed to have Stargardt macular dystrophy by clinical criteria were studied with a scanning laser ophthalmoscope (SLO) comparing argon laser blue (488 nm), green (514), helium-neon laser red (633 nm) and infrared diode laser (780 nm) images for the presence or absence of macular pigment in the fovea. Fifteen of the patients were screened for mutations in the ABCR gene. Eyes were graded into three categories: those without foveal macular pigment, those with partial pigment and those with normal amounts of macular pigment. These categories were compared with visual acuity determined by the Snellen chart.


RESULTS
All patients with a visual acuity of 20/200 or worse had no macular pigment in the fovea. All patients with visual acuity of 20/40 or better had a normal amount of macular pigment in the fovea. Patients with partial macular pigment had intermediary acuity values except for two eyes, one with 20/20 and another with 20/200 acuity. Infrared light revealed more retinal abnormalities than blue light at early stages of the disease.


CONCLUSIONS
Foveal macular pigment is related to foveal cone acuity in Stargardt macular dystrophy and may be a marker for the presence of foveal cones. Infrared light is a sensitive monitor of early Stargardt macular dystrophy.
Zhang
Xinyuan
X
Department of Ophthalmology, Columbia University, New York, NY 10032, USA.
Hargitai
János
J
Tammur
Jaana
J
Hutchinson
Amy
A
Allikmets
Rando
R
Chang
Stanley
S
Gouras
Peter
P
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005584:Fovea Centralis / Q000378:metabolism; D006801:Humans; D007834:Lasers; D008266:Macula Lutea / Q000378:metabolism* / Q000473:pathology; D008268:Macular Degeneration / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009887:Ophthalmoscopy / Q000379:methods; D012016:Reference Values; D012168:Retinal Pigments / Q000378:metabolism*; D014792:Visual Acuity
D012168:Retinal Pigments