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Case 17

Patient Presentation: A 24-year-old male with sickle cell disease (HbSS) presented to ophthalmology clinic for his annual eye examination. He does not endorse any visual complaints. On examination, visual acuity is 20/20 in both eyes. Slit lamp examination and fundus examination were normal with no obvious signs of sickle cell retinopathy. An OCT macula was performed demonstrating the following:



Retina 17-1.png
Retina 17-2.png

Question: What is the most notable abnormality in the OCT above?

Sickle cell maculopathy (labelled).png

Question: What is the significance of this OCT finding?

Question: What type of visual symptoms can patients with sickle cell-related temporal macular atrophy present with?

Learning Objectives:

1. Sickle cell maculopathy is characterized by focal thinning of the temporal macula.

2. The presence of temporal macular atrophy is suggestive of co-existing proliferative sickle cell retinopathy.

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