Evolution of a Retinal Hemorrhage in a Patient With Sickle Cell-Hemoglobin C Disease

In 1987, during a cross-sectional survey of patients with sickle cell disease on the island of Curaçao,¹ I documented a salmonpatch retinal hemorrhage in a 17-year-old black man with sickle cell-hemoglobin C disease. Part of the retinal hemorrhage had broken through into the vitreous; the intraretinal part of the hemorrhage was hemolyzed and pink. This hemorrhage originated from the (whitish) arteriole (Figure 1 and Figure 2).

During a follow-up visit in 1993, the hemorrhage had evolved into a black-sunburst chorioretinal scar (Figure 3 and Figure 4).

COMMENT  Sight-threatening complications in patients with sickle cell disease almost invariably arise from proliferative sickle cell retinopathy, which occurs mainly in patients with sickle cell-hemoglobin C disease or hemoglobin S-β-thalassemia. The majority of patients with homozygous sickle cell disease, sickle cell-hemoglobin C disease, or sickle cell-β-thalassemia, however, show sequelae of retinal hemorrhages that fortunately rarely affect vision.Hannon² coined the term salmon-pink