Retinal Microvascular Analysis Using Fundus Floruscein Angiography in Egyptian Sickle Cell Disease Patients

Abstract

Sickle cell disease (SCD) is a severe hematological disorder of chronic hemolysis, vascular injury and tissue ischemia impacting multiple organ systems. While homozygous hemoglobin S disease (HbSS) is associated with more severe clinical manifestations, HbSC disease is associated with more severe and earlier retinal disease. Ocular lesions result from stasis and occlusion of the small eye vessels by sickled erythrocytes. Vaso-occlusive disease of the retina can be responsible for nonproliferative and proliferative ocular changes (based on the absence or presence of vascular proliferation respectively). Our study included 30 patients with sickle cell disease; aging equal or more than 10 years with a mean age of 14.1± 4.02 years, and male-to-female ratio of 1.1:1; who regularly attended the Hematology clinic, children Hospital of Ain Shams University. All included patients were subjected to detailed medical history and thorough clinical examination with special emphasis on anthropometric measures, disease duration, history of sickling crises and avascular necrosis, transfusion history and iron chelation /hydroxyurea therapy. Laboratory investigations included CBC, baseline hemoglobin elecotrophoresis, and markers of hemolysis (LDH and indirect bilirubin). Mean serum ferritin level was calculated by collecting the serum ferritin done every 6 months in the last 2 years prior to the study. Trans-cranial Doppler (TCD) was done to all included patients.