Ocular manifestations in children and adolescents with sickle cell disease attending a Pediatric Hematology Unit in Damanhur Teaching Hospital

Abstract

Background Sickle cell disease (SCD) is the most common genetic disease worldwide. It is usually accompanied with painful crisis. Microvascular occlusions are common and affect different systems, among which is the ocular manifestations.Aim This study aimed to assess retinal changes in patients with SCD and its correlation with hematological parameters and if there is a link between a pretransfusion hemoglobin level and ocular manifestations or not.Patients and methods This study was conducted in the Pediatric Hematology Unit of Damanhur Teaching Hospital including patients coming regularly to sickle cell OPD for follow-up, those who were admitted for sickle cell crisis in internal medicine ward, as well as obstetrics/gynecology patients who were found to be sickle cell positive (SS pattern). In this cross-sectional study, 30 steady-state patients (60 eyes) aged 2–26 years (22 children and eight young adults) with an established diagnosis of SCD (16 with homozygous SS and 14 with S/b thalassemia) underwent complete ophthalmic examination with dilated fundoscopy. Hematologic investigations and ophthalmic investigations (visual acuity, slit-lamp biomicroscopy, and fundoscopy) were done and compared. For statistical analysis, SPSS software was used.Results The study showed that 70% of the patients with SCD with hemoglobin less than or equal to 7 g or less had ocular abnormalities in the form of nonproliferative retinopathy, proliferative retinopathy, and refractive errors.Recommendations Based on these observations, we recommend that all children with SCD (SS, S/B) should have regular ophthalmic examination including fundoscopic examination to screen for sickle cell retinopathy beginning at the age of 12 years.