Which optic disc findings are characteristic of hemoglobinopathy retinopathy?

Updated: Sep 03, 2019
  • Author: Brian A Phillpotts, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Vascular changes in the optic disc can be seen secondary to intravascular occlusions. These intravascular occlusions primarily affect the small vessels on the surface of the optic disc.

These lesions appear as dark red spots or clumps on the nerve head, often called the disc sign of sickling. The proposed mechanism for this sign is the transient plugging of sickled (deoxygenated) erythrocytes.

Fluorescein angiography (FA) reveals segments of linear or Y-configuration of hypofluorescence that correspond to the dark red spots. Blood flow through these vessels (as seen on angiography) is not impaired. [2] Like conjunctival vascular changes, these lesions are transient and do not produce any appreciable visual symptoms.

Optic nerve neovascularization is a rare complication of hemoglobinopathies.

Optic disc changes appear to be more common in patients with sickle cell anemia than in those with sickle cell C disease or sickle cell-thalassemia disease.

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