To evaluate the presence and progression of maculopathy in patients with sickle cell disease (SCD) using Optical Coherence Tomography (OCT) and OCT-Angiography (OCTA), and to identify clinical/laboratory risk factors for progression during follow-up.
Complete ophthalmic examination, including fundoscopy and macular SD-OCT/OCTA scans, was performed in consecutive SCD-patients (HbSS/HbSβ0/HbSβ+/HbSC genotype) during baseline and follow-up visits. SCR stage was based on fundoscopy instead of the Goldberg classification, since fluorescein angiography was not routinely used. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records.
106 eyes of 60 patients were analyzed. The median follow-up period was 34.5 months (range 8-70, IQR 25-55). Macular thinning was present in 41 eyes (38.7%) at baseline and in 52 eyes (49.1%) at follow-up. Progression of macular thinning was observed in 25.5% (27/106) of the eyes and SCR progression in 15.1% (16/106) of the eyes. Predictors for progression of macular thinning were proliferative retinopathy (adjusted OR (aOR) 3.40, p=0.024), lower vessel density in the superior capillary plexus of the inferior parafoveal subfield (aOR 0.88, p=0.003) and higher vessel density in the deep capillary plexus of the inferior parafoveal subfield (aOR 1.17, p=0.001). No association was found between progression of macular thinning and worsening of other organ damage, SCR progression, ocular complications or laser treatment.
SCD-related maculopathy progresses in many patients without impairing visual acuity during short-term follow-up. Progression of maculopathy is correlated with proliferative retinopathy and vessel densities in inferior parafoveal subfields. Further research is needed to elucidate functional consequences of macular changes.