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

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.