Which macular 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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Sickle cell retinopathy commonly occurs in the periphery, but macular changes also have been well documented.

Macular changes or sickling maculopathy can manifest acutely or chronically, occurring in patients with sickle cell disease, sickle cell C disease, and sickle cell-thalassemia disease.

Acute sickling maculopathy generally arises from acute vascular occlusion to the retina.

Involved vessels include the central retinal artery and its branches.

Acute vascular occlusion, although infrequent, can lead to acute retinal ischemia with subsequent infarction.

Acute retinal infarction may result in the complete loss of vision or can lead to central or paracentral scotomas, which may become incapacitating.

Chronic sickling maculopathy is more common and can be seen in up to 30% of the sickle cell C disease population. Clinically, signs of chronic sickling maculopathy are difficult to detect because these changes represent architectural alterations of the fine macular vasculature. Since these changes are insidious, conduct a thorough ocular examination.

Clinically, macular depression may be seen. This results when thinning and atrophy of the retina occurs secondary to retinal ischemia. This concave-shaped lesion, located next to the macula, presents on retinal biomicroscopy as a dark circle with a bright central reflex.

Although uncommonly seen, another PSR-associated sign is the macula hole. Predisposing factors are traction on the macula and vasoocclusive events. Traction on the macula results from neovascularization in the periphery and in the optic nerve. Vasoocclusive events in the macular, peripapillary, and perifoveal vessels (ie, capillaries) lead to local ischemia, infarction, retinal thinning, atrophy, and, ultimately, macula hole formation. Other signs include microaneurysms, enlarged segments of terminal arterioles, hairpin-shaped vascular loops, and an abnormal foveal avascular zone.

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