Ocular Complications in Adults with Sickle Cell Disease in Lagos – A Comparative Study

Abstract

Abstract Introduction: Sickle cell disease has been associated with ocular complications in several studies. However, it has not often been compared with nonhemoglobinopathy controls. Aim: The aim of this study was to determine the ocular complications of sickle cell disease patients compared with controls in Lagos. Study Design: This was an open-label comparative study. Materials and Methods: Consecutive consenting confirmed sickle cell disease patients with hemoglobin SS and hemoglobin SC (HbSS and HbSC) were recruited, as were age- and sex-matched hemoglobin AA (HbAA) (non-HbSS/HbSC) controls. All had genotype screened, interviewed, and examined. Systemic comorbidities (diabetes, hypertension, human immunodeficiency virus, etc.) were excluded. P <0.05 was considered statistically significant. Results: One hundred Non hemoglobinopathy controls HbAA versus 100 Sickle cell cases (85 HbSS plus 15 HbSC) were recruited. The mean age was similar in both cases (26.85 years) and controls (27.55 years). There were 118 male versus 82 female respondents. Cases (77%) had yellow eye discoloration compared to zero controls. Cases (63%) had conjunctival sickle sign, none in controls. Cases (17%) had retinal vascular tortuosity and only 1% of controls. Potentially blinding proliferative sickle cell retinopathy (PSR) was found in 20% of HbSC and in 7.1% of HbSS patients, none in controls. There was a significant association with age and ptosis in cases (P = 0.001), no significant association between gender and ocular findings. Conclusions: Ophthalmic manifestations occur in sickle cell disease patients. Prompt initial and periodic ophthalmic evaluation is recommended in all hemoglobin HbSS and HbSC patients to identify lesions amenable to treatment.