Ophthalmologic complications in hemoglobinopathies

The various forms of sickle cell disease share the common feature of an abnormal globin chain that, under certain conditions such as hypoxia, results in the sickling of red blood cells and obstruction of blood vessels. The ophthalmic manifestations of sickle cell disease are present in various segments of the eye, which include the conjunctiva, iris, retina, and optic nerve. Patients with SC disease and SB thalassemia tend to have more severe ophthalmic complications than their counterparts with the more severe systemic form of sickle cell disease--SS disease. It is important for patients with sickle cell disease to have periodic examinations by ophthalmologists, because the early identification of peripheral retinal disease can be adequately treated, with preservation of vision. Without treatment, peripheral retinal changes can lead to retinal detachments and vitreous hemorrhages, which account for the majority of visual impairment in patients with sickle cell disease.