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CHOROIDAL VASCULARITY INDEX, RETINAL VASCULARITY, AND HEMOGLOBIN LEVELS IN PEDIATRIC SICKLE CELL MACULOPATHY

Celine Chaaya1,2, Sandra Hoyek1,2, Francisco Altamirano2

  • 1Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts.

Retina (Philadelphia, Pa.)
|October 15, 2025
PubMed

Insights

Pediatric sickle cell disease (SCD) patients show reduced choroidal vascularity index (CVI), linked to lower retinal vessel density and hemoglobin levels, suggesting choroidal ischemia in sickle cell maculopathy.

Area of Science:

  • Ophthalmology
  • Hematology
  • Pediatrics

Background:

  • Sickle cell disease (SCD) can affect the eyes, potentially leading to complications like sickle cell maculopathy.
  • The choroidal vascularity index (CVI) is a measure of vascularity in the choroid, a layer of blood vessels in the eye.
  • Understanding ocular changes in pediatric SCD is crucial for early detection and management.

Purpose of the Study:

  • To evaluate the choroidal vascularity index (CVI) in children with sickle cell disease (SCD).
  • To assess the association between CVI, retinal thickness, and retinal vasculature in pediatric SCD patients.
  • To compare CVI in pediatric SCD patients with healthy controls.

Main Methods:

  • Retrospective case series comparing pediatric SCD patients with age-matched healthy controls.
  • Automated machine learning algorithm used for Choroidal Vascularity Index (CVI) measurement.
  • Retinal assessments included total retinal thickness and vessel density (VD).

Main Results:

  • Pediatric SCD patients had a significantly lower mean CVI (54%) compared to healthy controls (69%).
  • Lower CVI in SCD eyes was associated with reduced inferotemporal retinal vessel density (VD) and increased retinal thickness in specific macular areas.
  • Eyes with lower CVI also showed significantly lower mean hemoglobin (Hgb) levels.

Conclusions:

  • A decreased CVI in pediatric SCD patients suggests potential choroidal ischemia contributing to sickle cell maculopathy.
  • CVI may serve as a valuable biomarker for assessing systemic disease activity and ocular involvement in pediatric SCD.
  • Further research can explore CVI's role in monitoring disease progression and treatment efficacy.
Abstract

Keywords:
choroidal vascularity indexoptical coherence tomographysystemic findings

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