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Patient Presentation: A 23-year-old obese female was diagnosed with idiopathic intracranial hypertension (IIH) and referred to neurosurgery for ventriculoperitoneal shunt. A baseline ocular examination was performed prior to the procedure.

On examination, vision was 20/200 in the right eye, and 20/40 in the left eye. There was a right relative afferent pupillary defect. Slit lamp examination was normal.

A dilated fundus examination was performed demonstrating the following:

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Retina

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:

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

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Question: What is the significance of this OCT finding?

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

A. Asymptomatic

C. Impaired colour vision and contrast sensitivity

D. All of the above

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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