Detecting sickle cell retinopathy

Evaluation of the macula with optical coherence tomography (OCT) should be performed as a routine screening test for retinopathy in paediatric patients with sickle cell disease, said Léopoldine Lequeux MD at the World Society of Paediatric Ophthalmology & Strabismus Subspecialty Day in Paris, France. Dr Lequeux’s recommendation was based on findings from a study in which 24 patients aged 6-to-15 years (mean 11 years) with sickle cell disease were evaluated for macular abnormalities using spectral domain (SD) OCT and OCT angiography, both with the Topcon Triton system. The OCT imaging revealed that atrophic maculopathy caused by ischaemic involvement was common in the examined paediatric population. Furthermore, there was a strong correlation between the presence of maculopathy and retinopathy – maculopathy was present in 93% of eyes with retinopathy and absent in 96% of eyes without retinopathy. “Screening for sickle cell retinopathy typically includes a fundus examination, supplemented by fluorescein angiography if necessary. We added examination of the macula with macular mapping using SD OCT and OCT angiography,” said Dr Lequeux, Centre Ophtalmologie Rive Gauche, Toulouse, France. “These findings suggest that macular OCT may be used instead of the more restrictive fundus examination as a screening test for sickle cell retinopathy.” Dr Lequeux noted that peripheral retinal ischaemia involvement of sickle cell disease and neovascular complications of sickle cell retinopathy are well known. Because ischaemic maculopathy has also been described, she and her colleagues were motivated to use OCT-based imaging to look for macular abnormalities and investigate their correspondence with peripheral retinal changes. Among the 24 patients in the study, eight (33%) had retinopathy. The retinopathy was early stage (stage I or II Goldberg classification) in all cases and more common among patients with the SC genotype of sickle cell disease (60%) than in those with the SS form (29%). Two patients had S-Beta thalassemia genotype sickle cell disease, of which neither had retinopathy. Macular abnormalities, mostly temporal retinal thinning, were identified with SD OCT in 33% of the 24 patients. The macular abnormalities did not translate to angiographic abnormalities, nor were they associated with any symptoms, said Dr Lequeux. OCT angiography identified macular ischaemia, showing deep capillary perfusion abnormalities in the same region as the macular atrophic changes found on SD OCT. Léopoldine Lequeux: drlequeux@gmail.com