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F. Y. Chau, Q. V. Hoang, J. I. Lim; Rates of Focal Thinning in the Macula or Temporal Retina in Sickle Cell Genotypes by Spectral Domain Optical Coherence Tomography. Invest. Ophthalmol. Vis. Sci. 2010;51(13):3554.
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Sickle cell anemia is characterized by infarctions resulting in systemic crises as well as various stages of sickle retinopathy. Infarctions may occur in the macula or surrounding retina and cause changes in retinal architecture to subtle degrees that may now be detected by spectral domain optical coherence tomography (SDOCT). Using SDOCT, this study investigated the frequency of focal thinning in the macula or temporal retina in various genotypes of sickle cell anemia patients.
59 patients with sickle cell anemia of various genotypes (SS, SC, SThal) and 10 control patients without sickle were scanned by SDOCT (Heidelberg Spectralis). Regions of focal thinning were identified as an abrupt, asymmetric decrease in total retinal thickness. Rates of focal thinning in the macula or temporal retina were calculated.
59 total sickle cell patients were imaged. Focal macular or temporal retinal thinning occurred in 7 of 14 (50%) SC patients, 21 of 39 (54%) SS patients, and 4 of 6 (67%) SThal patients. No control patients had any regions of focal thinning. Inner retinal layers were most commonly affected in regions of focal thinning, likely representing ischemic retinal atrophy from occlusions of the retinal circulation.The frequency of focal retinal thinning was stratified by individual eye and its stage of retinopathy. Two blind eyes were excluded.For SC patients, focal retinal thinning occurred in0 of 1 (0%) eye without retinopathy,0 of 1 (0%) Stage 1 eye,1 of 11 (9%) Stage 2 eyes,7 of 12 (58%) Stage 3 eyes,1 of 2 (50%) Stage 4 eyes, and1 of 1 (100%) Stage 5 eye.For SS patients, focal retinal thinning occurred in3 of 14 (21%) eyes without retinopathy,2 of 8 (25%) Stage 1 eyes,21 of 44 (48%) Stage 2 eyes,5 of 8 (63%) Stage 3 eyes,1 of 1 (100%) Stage 4 eye, and1 of 1 (100%) Stage 5 eye.For SThal patients, focal retinal thinning occurred in0 of 2 (0%) eyes without retinopathy,0 of 2 (0%) Stage 1 eyes,3 of 4 (75%) Stage 2 eyes, and4 of 4 (100%) Stage 3 eyes.Focal thinning in the macula or temporal retina thus tended to occur more often with increasing stage of sickle retinopathy.
Focal thinning in the macula or temporal retina is a common finding in sickle cell patients and tends to occur more frequently with increasing stage of sickle retinopathy. Regions of focal retinal thinning can be identified and quantified by spectral domain optical coherence tomography.
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