Ocular Manifestations of Sickle Cell Disease With an Emphasis on Retinal Involvement

Background This study aimed to evaluate the spectrum of ocular manifestations in sickle cell disease (SCD) patients, with a particular emphasis on retinal involvement, to improve early diagnosis and management strategies. Methodology A cross-sectional, observational study was conducted over one year at Assam Medical College and Hospital, Dibrugarh, recruiting 96 SCD patients aged 10 and above. Comprehensive ophthalmological evaluations included best-corrected visual acuity, slit-lamp biomicroscopy, direct/indirect ophthalmoscopy, spectral-domain optical coherence tomography (OCT), and, in selected cases, fundus fluorescein angiography. Sickle cell retinopathy (SCR) was graded using Goldberg's classification. Results The mean age of the participants was 31.00 ± 13.89 years, with 52 (54.17%) males and 71 (76.04%) from the tea tribe community. Systemic features included pallor in 85 (88.54%) and generalized weakness in 83 (86.46%) participants. Visual acuity of 6/6 to 6/9 was found in 32 (33.33%) (right eye) and 33 (34.38%) (left eye) participants. Anterior segment involvement (corkscrew vessels) was noted in 50 (52.08%) participants, and posterior segment changes were noted in 51 (53.13%) participants. Non-proliferative SCR was present in 46 (47.92%) and proliferative SCR in 5 (5.21%) participants. OCT revealed vitreomacular traction, epiretinal membranes, and full-thickness macular holes. Patient age correlated with retinopathy severity (R² = 0.76, p < 0.01), and systemic severity correlated with retinopathy (R² = 0.94, p < 0.001). Conclusions SCD patients exhibit significant ocular manifestations, particularly retinal pathology, which correlates with age and systemic disease severity. Early and routine ophthalmologic screening is crucial for timely management and preventing vision loss.