Incidence of ocular abnormalities in patients with sickle hemoglobinopathies

We examined 110 consecutive unselected patients with SS, SC, or Sthal sickle hemoglobinopathies to determine the prevalence of various retinal lesions within each group. The most prevalent retinal abnormality was the black sunburst, occurring in 46% of SS patients, 63% of SC patients, and 37% of Sthal patients. Our data also suggest that retinal lesions become more prevalent up to the fifth decade of life. Included in our series was an eight-year-old SS hemoglobin patient who presented with a dense vitreous hemorrhage secondary to a large salmon patch that had bled into the vitreous, reducing her vision to 20/200.