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Correlations Between Visual Field Defects and Macular Thinning in Sickle Cell Disease.

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Sickle cell disease (SCD) maculopathy, characterized by temporal thinning, correlates with visual field defects, even with normal visual acuity. This suggests vascular dysfunction contributes to vision impairment in SCD patients.

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Area of Science:

  • Ophthalmology
  • Hematology
  • Medical Imaging

Background:

  • Sickle cell disease (SCD) can lead to ocular complications, including maculopathy.
  • Maculopathy in SCD may impact visual function, but its precise relationship with visual field defects is not fully understood.

Purpose of the Study:

  • To investigate the correlation between visual field defects from automatic perimetry and macular temporal thinning detected by optical coherence tomography (OCT) in patients with SCD.
  • To define the impact of SCD-related maculopathy on visual function.

Main Methods:

  • A retrospective cross-sectional study involving 58 patients with SCD was conducted.
  • Ophthalmological examinations included automatic perimetry and OCT.
  • Correlation analysis was performed between macular thickness and visual field defects in ETDRS quadrants.

Main Results:

  • Normal visual acuity was observed in 94.5% of eyes.
  • 30% of eyes exhibited visual field defects.
  • Significant correlation found between macular thinning on OCT and decreased visual field mean defect in the temporal quadrant (P < 0.001).
  • Macular thickness was reduced in temporal quadrants in SS genotype patients and in inner/outer temporal quadrants in other genotypes.

Conclusions:

  • Macular temporal thinning in SCD is associated with visual field defects, particularly in SS genotypes, despite normal visual acuity.
  • Findings suggest a primary vascular dysfunction, rather than a neurogenic mechanism, underlies SCD maculopathy.