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Management

Screening

Ocular Imaging with SDOCT has been shown to be helpful in identification of patients with retinal
thinning related to sickle cell retinopathy. Retinal thinning has been associated with decreased retinal
sensitivity as shown by microperimetry

Age, stage of retinopathy, hemoglobin SS subtype and some systemic conditions are associated with
more severe retinal thinning

OCT angiography (OCTA) has also shown significant changes even in children without structural damage

There is no therapy at present for non-proliferative sickle cell retinopathy.

Therapy for PSR stage 3 is to prevent bleeding and development of retinal detachment. Complete
ophthalmic examination at least yearly is recommended for sickle cell patients (SC, SS, S-thal, etc).
SDOCT and OCTA imaging add to the clinical staging of the disease and may be performed. Baseline
fluorescein angiography may be performed to examine blood flow in the retina and choroid as needed

Systemic therapy

hydroxycarbamide treatment has been demonstrated to have a beneficial effect in children in

preventing sickle cell retinopathy.[20] Similarly, reducing HbSS red blood cells by exchange transfusion has
also shown to be beneficial.[21][22]Hyperbaric oxygen therapy may reverse the pathology and improve
visual acuity.[23] [24] Hydroxyurea is often used and is believed to increase the levels of HgbF.

Anti-VEGF

As complications of PSR arise, intravitreal administration of anti-vascular endothelial growth factor (anti-
VEGF) therapy[25] and/or scatter laser photocoagulation[26] may lead to regression of neovascularization

Laser

Scatter laser for treatment of seafans has been shown to be effective and safer than feeder vessel
techniques

Scatter laser to surround seafans is the current treatment for seafans

n the event of vitreous hemorrhage, patients need to be examined at regular intervals to document
clearance and improvement in visual acuity.