Sickle Cell Retinopathy

It is the most important eye complication in sickle cell disease (SCD). It’s important to talk about it because it can lead to partial or complete loss of vision

Mind you, there are other eye disease that can affect someone living with SCD that are not directly caused by SCD, such as physical injuries to the eye, or refractive errors.

Also there are other eye disease in SCD that do not involve the retina, such as iris atrophy, conjunctiva vaso occlusion..Specifically we talk about Retina diseases in SCD

The retina

 The innermost light sensitive area at the back of the eye. Vision is impossible without health retina. The retina is like the lens in the camera. It captures the image and sends to the brain for processing, so you can see 😊

 Retinopathy: Disease of the retina

Sickle retinopathy: Diseases of the retina caused by SCD. There are 2 types : Non proliferative retinopathy(NPR) and Proliferative Retinopathy(PR).

Simply put, Proliferative Retinopathy involves formation of new blood vessels(proliferation); in contrast to NPR. NPR is mild, while PR can be severe (Stages 1 – 5), resulting in complete loss of vision.


 Retinopathy is not as common as bone pain crisis and some other complications but it does happen. Typically, it is ASYMPTOMATIC in the early stages, as such requires surveillance in persons at risk.

It affects about 12% of persons with SCD( 1 in 10 persons) and it presents with varying degrees of severity. Higher risk occurs in Haemoglobin SC, lower foetal haemoglobin, raised Packed cell volume.

CAUSE: chronic hypoxia (low oxygen supply) in the eye + occlusion of retinal vessels by sickle cells

 CLINICAL PRESENTATION(Symptoms and signs):

Acute- sudden loss of vision ( Central retinal artery occlusion)

Gradual: Patients may see flashes, floaters or dark shadows, visual loss and complete blindness.

Eye Examination by Specialist ( Ophthalmologist). That involves visual acuity, papillary reactivity, slit lamp exam, dilated fundoscopy, fluorescein angiography, etc

-comma shaped vessels/conjunctiva

– new blood vessels in retina

-retinal hemorrhages



Primary prevention: Optimal Care which could include usual care, hydroxyurea blood transfusions and stem cell transplant

Early Diagnosis: Annual eye examination and regular follow up with your consultant haematologist


– Laser photocoagulation

– Micro surgery ( non clearing vitreous Haemorhage and retinal detachments)

– Anti-VEGF

– Other systemic treatments ( blood transfusions, Hydroxyurea)

Treatment Expert: Consultant Ophthalmologist ( Doctors that treat eye diseases).

Short simple presentation and we can take a lot of questions, so we can learn and share knowledge

 Good evening Doc. Those who already have eye defects, and already use prescribed glasses are they likely to have this Retinopathy?

 Does this affect everyone Sickle Cell Warrior

 Can it be corrected?

 What causes occlusion of retinal vessels by sickle cells

The risk in warriors using glasses is independent of the glasses. Prescribed glasses will not contribute to Retinopathy. Refraction errors has to do with the lens and eye muscles

 Mind you, Not every warrior is affected. We realy do not have strong  data for our nation, but from other countries about 1 in 10 warriors will be affected and it’s more common in Haemoglobin SC

 Yes, it is reversible in early stages, at advanced stage there is bleeding into the retina and detachment of the retina  … at that stage , it is more difficult to handle, but options of vitrectomy and other micro surgeries is there

 When  the red cell becomes sickle, it is less soluble, more rigid, more sticky and less flexible, so it gets stuck within small blood vessels in the retina and blocks it.

So how do we prevent this?

As much as possible, prevention has to do with optimal care for sickle cell disease as a whole, early detection  through regular check and treatment  before serious complications set in

 If that’s all the questions, we will have to let Doc go so he attends to other things.

So Doc. are you saying people with hemoglobin SS, are free from retinopathy?

 Nope. But it is more common and tends to be more severe in SC

Thank you Dr. Demola. We appreciate you sir

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