Abstract • The disc sign is the presence of dark red spots on the optic disc of patients with a sickling hemoglobinopathy. The dark spots appear to be plugs of deoxygenated erythrocytes in small surface disc vessels. The occlusions, which are transient and do not produce clinically detectable visual impairment, were seen most often in patients with homozygous sickle cell anemia and may be the result of clogging of small vessels by irreversibly sickled erythrocytes. References 1. Serjeant GR: The Clinical Features of Sickle Cell Disease . New York, American Elsevier Publishing Co Inc, 1974. 2. Paton D: The conjunctival sign of sickle cell disease . Arch Ophthalmol 66:90-94, 1961.Crossref 3. Goodman G, von Sallmann L, Holland MG: Ocular manifestations of sickle-cell disease . Arch Ophthalmol 58:655-682, 1957.Crossref 4. Serjeant GR, Serjeant BE, Condon PI: The conjunctival sign in sickle cell anemia . JAMA 219:1428-1431, 1972.Crossref 5. Nagpal KC, Asdourian GK, Goldbaum MH, et al: The conjunctival sign, hemoglobin S, and irreversibly sickled erythrocytes . Arch Ophthalmol 95:808-811, 1977.Crossref 6. Condon PI, Serjeant GR, Ikeda H: Unusual chorioretinal degeneration in sickle cell disease: Possible sequelae of posterior ciliary vessel occlusion . Br J Ophthalmol 57:81-88, 1973.Crossref 7. Dizon R, Jampol L, Goldberg M: Acute posterior ciliary artery occlusion in sickle cell disease . Invest Ophthalmol Visual Sci (April (suppl) ):160-161, 1978. 8. Galinos S, Rabb MF, Goldberg MF, et al: Hemoglobin SC disease and iris atrophy . Am J Ophthalmol 75:421-425, 1973. 9. Welch RB, Goldberg MF: Sickle-cell hemoglobin and its relation to fundus abnormality . Arch Ophthalmol 75:353-362, 1966.Crossref 10. Galinos SO, Asdourian GK, Woolfe MB, et al: Spontaneous remodeling of the peripheral retinal vasculature in sickling disorders . Am J Ophthalmol 79:853-870, 1975. 11. Stevens TS, Busse B, Lee C-B, et al: Sickling hemoglobinopathies: Macular and perimacular vascular abnormalities . Arch Ophthalmol 92:455-463, 1974.Crossref 12. Asdourian GK, Nagpal KC, Busse B, et al: Macular and perimacular vascular remodelling in sickling haemoglobinopathies . Br J Ophthalmol 60:431-453, 1976.Crossref 13. Henkind P: Radial peripapillary capillaries of the retina: I. Anatomy: human and comparative . Br J Ophthalmol 51:115-123, 1967.Crossref 14. Goldbaum MH: Retinal depression sign: A newly observed sign indicating a small retinal infarct. Am J Ophthalmol, to be published. 15. Condon PI, Serjeant GR: Ocular findings in homozygous sickle cell anemia in Jamaica . Am J Ophthalmol 73:533-543, 1972. 16. Condon PI, Serjeant GR: Ocular findings in hemoglobin SC disease in Jamaica . Am J Ophthalmol 74:921-931, 1972. 17. Condon PI, Serjeant GR: Ocular findings in sickle cell thalassemia in Jamaica . Am J Ophthalmol 74:1105-1109, 1972.
Archives of Ophthalmology – American Medical Association
Published: Sep 1, 1978
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