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Chronic eye complications in sickle cell disease: Everyone has a role to play.

We said in the previous articles that the management of sickle cell disease requires a multidisciplinary approach with the primary clinician at the centre of it. Chronic eye complications strictly require the services of an ophthalmologist. However, the ophthalmologist will not find the patient along the dusty streets of Kampala nor the muddy feeder road in Apac.

The forefront clinician will have to identify such a patient and either refer them or send in a consultation request to the ophthalmologist. Let’s get this started.

Two major chronic eye complications are present among people living with sickle cell disease:
-proliferative sickle retinopathy and
-vitreous haemorrhage.

They affect about 50% of the individuals with sickle cell disease, especially HbSC and HbSS.
Proliferative sickle retinopathy is associated with a substantial loss of vision. It occurs earlier in HbSC than HbSS.

Related article: Acute ocular complications in sickle cell disease are always medical emergencies: Prompt management is crucial.

When the sickle red cells occlude the retinal arterioles, ischaemia results: new blood vessels form.
The blood vessels are often very friable and easily worn by tear due to the vitreoretinal traction forces that also lead to haemorrhage. These newly formed blood vessels appear bright red. When they are infarcted, the white structures that appear as ‘sea-fans’. The repetitiveness of these processes culminates in proliferative retinopathy.

PSR has five stages. We use direct and indirect ophthalmoscopy, slit-lamp biomicroscopy and fluorescein angiography to identify them.

In about 32% of eyes, proliferative sickle retinopathy may regress. In a few others, the sea-fan formation may not progress to vitreous haemorrhage.

Upon consultation with an ophthalmologist, they can use laser photocoagulation to treat proliferative sickle retinopathy. Or surgical interventions like vitrectomy for severe vitreous haemorrhage can be performed.

We adopted the following recommendations from the report on evidence-based management of sickle cell disease.

1. Refer persons of all ages with PSR for evaluation and possible laser photocoagulation therapy.
2. Refer children and adults with vitreoretinal complications of PSR refractory to medical treatment. An ophthalmologist must evaluate them for possible vitrectomy.

The article brings us to the end of the article series about the chronic complications of sickle cell disease. I hope you’ve benefited from this knowledge, and if so, share it with a friend.


MBChB (MUK), Graduate Fellow, Department of Physiology, Makerere University Founder and Content Creator Peer reviewer, Associate Editor

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