Background: Homozygous sickle cell disease is prevalent in tribal population of South Gujarat. Aim: To determine prevalence of different ocular manifestations in pediatric patients with homozygous sickle cell disease in South Gujarat. Setting and Design: Cross sectional study at tertiary level government hospital and primary health centers. Methods: 125 children with homozygous sickle cell disease, who fulfilled the criteria were enrolled, were enrolled in the study during a period from December 2015 to June 2017. We collected data on demography, ocular history, hospitalization history and complete eye examination. We calculated systemic severity index. Statistical analysis used: We used SPSS version 20. p value ≤ 0.05 was considered to be statistically significant. Results: Majority of children were from age group 11–18 years (68.8%) and belonged to schedule tribe with almost equal gender distribution; M: F = 1:1.08. Ocular abnormalities were observed in 43 (34.4%) patients. Anterior segment presentations were: Conjuctival sign-21 (16.8%), Iris atrophy-1 (0.8%). Posterior segment signs were: Retinal venous tortuosity-25 (20%), High cup: Disc ratio-3 (2.4%), Vitreous hemorrhage (Grade 4 Proliferative Central Retinopathy) -4 patients (3.2%), Salmon patch hemorrhage-2 (1.6%), Angioid streaks-2 (1.6%), Drusen-1 (0.8%), Central retinal artery occlusion-1 (0.8%). Ocular manifestations were found to be statistically significant in patients with systemic severity index between 0.8- 1. Blindness was found in 4% cases. Conclusion: Mild ocular morbidities are found in children having homozygous sickle cell disease in most vulnerable communities of South Gujarat. Regular eye check-up is recommended in children having high systemic severity index for early diagnosis and treatment of potential blinding conditions.

Keywords: Homozygous sickle cell disease; Systemic severity index; Venous tortuosity; Conjuctival sickling sign; Vitreous hemorrhage.