Ocular manifestations of sickle hemoglobinopathies

Abstract

Sickle cell disease is a hereditary disorder that is characterized by the production of structurally abnormal hemoglobin molecules. Clinical manifestations depend upon the amount and types of abnormal hemoglobin present. This paper reviews the genetic and molecular basis of sickle hemoglobinopathies and thalassemias including sickle cell anemia, SC disease, sickle cell-Beta Thalassemia and sickle trait. The systemic and ocular manifestations of these diseases are presented. Treatment regimens pertaining to hyphema, proliferative retinopathy, vitreous hemorrhage and retinal detachment are also discussed.