Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 May 1;45(5):928-938.
doi: 10.1097/IAE.0000000000004393. Epub 2025 Jan 2.

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

Rajani P Brandsen  1   2 , Bart J Biemond  2 , Erfan Nur  2   3 , Jennifer M Chang-Wolf  1 , Reinier O Schlingemann  1   4 , Roselie M H Diederen  1
Affiliations

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

Rajani P Brandsen et al. Retina. .

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.

PubMed Disclaimer

References

    1. Asdourian GK, Nagpal KC, Busse B, et al. Macular and perimacular vascular remodelling sickling haemoglobinopathies. Br J Ophthalmol 1976;60:431–453.
    1. Minvielle W, Caillaux V, Cohen SY, et al. Macular microangiopathy in sickle cell disease using optical coherence tomography angiography. Am J Ophthalmol 2016;164:137–144.e1.
    1. Brasileiro F, Martins TT, Campos SB, et al. Macular and peripapillary spectral domain optical coherence tomography changes in sickle cell retinopathy. Retina 2015;35:257–263.
    1. Han IC, Tadarati M, Scott AW. Macular vascular abnormalities identified by optical coherence tomographic angiography in patients with sickle cell disease. JAMA Ophthalmol 2015;133:1337–1340.
    1. Hoang QV, Chau FY, Shahidi M, Lim JI. Central macular splaying and outer retinal thinning in asymptomatic sickle cell patients by spectral-domain optical coherence tomography. Am J Ophthalmol 2011;151:990–994.e1.

Publication types