Sickle Cell Disease

  • Inherited disorder in which red blood cells are deformed and clog small arteries
  • Common systemic manifestations: episodes of arm, leg, back, and stomach pain
  • Common ophthalmic manifestations: hemorrhagic arteriolar occlusions in peripheral retina
  • Ophthalmic manifestations common in Sickle-Hemoglobin C Disease and Sickle-Thalassemia Disease, less common in Sickle Cell Disease, and rare in Sickle Cell Trait
  • Feared ophthalmic complications: vitreous hemorrhage, retinal detachment
  • Refer any patient with Sickle-Hemoglobin C Disease or Sickle-Thalassemia Disease for baseline ophthalmologic examination
  • Refer emergently patients with any variant of Sickle Cell Disease who suffer acute visual loss
  • Neglected retinopathy can lead to vitreous hemorrhage, retinal detachment, and blindness
  • Laser photocoagulation can interrupt this cascade if applied early