Sickle cell disease and the eye
- Eye and systemic disease
Sickled cells block small retinal vessels and can grow fragile new ones. A hyphema in sickle disease is high risk.
What it is
In sickle cell disease, sickled red cells block small vessels at the periphery of the retina. The ischaemic retina can grow new vessels shaped like sea fans, which bleed or pull the retina off. Proliferative retinopathy is most common in haemoglobin SC disease, not in the more severe SS disease. In the front of the eye, a small hyphema can raise eye pressure dangerously, even in sickle trait.
Eye findings
- Non-proliferative signs: salmon-patch haemorrhages, black sunburst scars, comma-shaped conjunctival vessels.
- Proliferative retinopathy: sea-fan new vessels at the peripheral retina, vitreous haemorrhage, detachment.
- Highest risk of proliferative retinopathy: haemoglobin SC disease.
- Angioid streaks can occur.
- Hyphema in sickle disease or trait: sickled cells block the drainage angle and the optic nerve tolerates pressure poorly.
Screening and follow-up
- Carbonic anhydrase inhibitors (acetazolamide) can promote sickling and should be avoided in sickle patients with hyphema.
- Every Black or mixed-ancestry patient with a traumatic hyphema should know their sickle status.
- Proliferative sickle retinopathy is treated with laser to the ischaemic retina.
Next step / Initial management
- Sickle cell disease: dilated retinal exams from about age 10, then periodically.
- Hyphema: sickle test if status unknown; same-day ophthalmology, close pressure control, early surgical washout if pressure stays high.
- Vitreous haemorrhage or detachment: same-day ophthalmology.
- Avoid dehydration and hypoxia, which promote sickling, during eye surgery.
Test yourself
Think of the answer, then tap the question to see it.
Which sickle genotype carries the highest risk of proliferative retinopathy?
Haemoglobin SC disease.
Why is a hyphema in sickle disease or trait dangerous?
Sickled cells block the drainage angle, pressure rises easily, and the optic nerve tolerates it poorly.
Which drug is avoided in sickle patients with hyphema?
Carbonic anhydrase inhibitors such as acetazolamide, which can promote sickling.
Connections
In Pharmacology1
Educational content; not a clinical guideline and no substitute for assessment by a health professional. For sudden visual loss, severe eye pain, a chemical burn or an eye injury, go to an emergency department at once.
Sources: Sickle Cell Anemia (StatPearls Publishing (NCBI Bookshelf), 2023); Hyphema (StatPearls Publishing (NCBI Bookshelf), 2026).
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Representative model, for educational purposes only.