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Updated: Feb 21, 2026

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Sickle cell disease and the eye.

Brian K Do1, Damien C Rodger

  • 1aUSC Roski Eye Institute, Keck School of Medicine bUSC Institute for Biomedical Therapeutics, University of Southern California, Los Angeles, California, USA.

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Recent literature shows sickle cell retinopathy (SCR) affects the macula early, with deep capillary plexus abnormalities and macular thinning even in asymptomatic patients. These findings challenge previous understandings of SCR progression.

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Area of Science:

  • Ophthalmology
  • Hematology
  • Vascular Biology

Background:

  • Sickle cell disease (SCD) is a group of inherited red blood cell disorders.
  • Ophthalmic manifestations of SCD include sickle cell retinopathy (SCR).
  • SCR is the most common ocular complication of SCD.

Purpose of the Study:

  • To review recent scientific literature on sickle cell retinopathy (SCR).
  • To specifically focus on the emerging understanding of sickle cell maculopathy.

Main Methods:

  • Literature review of recent studies on SCR and sickle cell maculopathy.
  • Analysis of findings from spectral-domain optical coherence tomography (SD-OCT) and OCT angiography (OCTA).

Main Results:

  • Macular perfusion abnormalities affect both superficial and deep capillary plexi in SCD patients.
  • Deep capillary plexus involvement is more common, linked to macular thinning.
  • Retinal changes occur even in asymptomatic individuals, differing from diabetic retinopathy and retinal vein occlusion.
  • Paracentral acute middle maculopathy is implicated in microvascular occlusion leading to macular thinning.

Conclusions:

  • Sickle cell disease causes diverse intraocular and orbital complications due to vaso-occlusive episodes.
  • SCR, though historically viewed as peripheral, significantly involves the macula.
  • Advanced imaging (SD-OCT, OCTA) reveals early and significant macular vascular changes in SCR, even before symptoms manifest.