Sickle Cell Disease

What is it?

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

Patient will not have visual symptoms unless retinal bleeding or detachment occurs
Indirect ophthalmoscopy discloses round orange patches in peripheral retina

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