LONGITUDINAL CHANGES OF MACULAR NEURODEGENERATIVE AND VASCULAR ABNORMALITIES ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN SICKLE CELL DISEASE

Abstract

Purpose: To evaluate the presence and progression of maculopathy in patients with sickle cell disease using optical coherence tomography and optical coherence tomography-angiography and to identify clinical/laboratory risk factors for progression during follow-up.

Methods: Complete ophthalmic examination, including fundoscopy and macular spectral-domain-optical coherence tomography/optical coherence tomography angiography scans, was performed in consecutive patients with sickle cell disease (HbSS/HbSβ 0 /HbSβ + /HbSC genotype) during baseline and follow-up visits. Sickle cell retinopathy stage was based on fundoscopy instead of the Goldberg classification, as fluorescein angiography was not routinely used. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records.

Results: One hundred and six eyes of 60 patients were analyzed. The median follow-up period was 34.5 months (range 8-70, interquartile range 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 sickle cell retinopathy progression in 15.1% (16/106) of the eyes. Predictors for the progression of macular thinning were proliferative retinopathy (adjusted odds ratio 3.40, P = 0.024), lower vessel density in the superior capillary plexus of the inferior parafoveal subfield (adjusted odds ratio 0.88, P = 0.003), and higher vessel density in the deep capillary plexus of the inferior parafoveal subfield (adjusted odds ratio 1.17, P = 0.001). No association was found between the progression of macular thinning and the worsening of other organ damage, sickle cell retinopathy progression, ocular complications, or laser treatment.

Conclusion: Sickle cell disease-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.

Keywords: OCTA; macular abnormalities; maculopathy; optical coherence tomography angiography; sickle cell disease; sickle cell maculopathy; sickle cell retinopathy.