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Iris Atrophy in Sickle Cell Disease

Iris Atrophy in Sickle Cell Disease

A 56-year-old man with sickle hemoglobin C (HbSC) disease presented to the ophthalmology clinic for a routine eye examination. He had a history of proliferative sickle cell retinopathy, which had been treated with laser photocoagulation. At the current presentation, he was asymptomatic. On examination, the corrected visual acuity was 20/20 in both eyes. Mild nuclear sclerosis of the lens and extensive iris atrophy were noted in the anterior segments of both eyes. Fibrotic retinal neovascularization that was consistent with treated and regressed retinopathy was also seen in both eyes. Sickle cell disease can affect many structures of the eye, and patients with the HbSC genotype are more susceptible to ocular complications than those with other genotypes. Iris atrophy is thought to be related to vaso-occlusion with infarction, and this finding on examination is closely associated with proliferative sickle cell retinopathy in the same eye. At a 6-month follow-up visit, the patient remained asymptomatic.

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