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Sickle cell retinopathy in young children in Jamaica
Insights
Sickle cell disease in children shows common retinal vessel abnormalities like arteriolar sheathing and peripheral closure. Retinal changes were more frequent in sickle cell (SS) disease but not statistically significant compared to sickle cell-haemoglobin C (SC) disease.
Area of Science:
- Ophthalmology
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) encompasses sickle cell (SS) disease and sickle cell-haemoglobin C (SC) disease, both potentially affecting ocular health.
- Pediatric SCD patients require regular ophthalmological monitoring to detect early-stage retinal complications.
Purpose of the Study:
- To investigate and compare the prevalence of retinal vascular abnormalities in children with SS disease versus SC disease.
- To identify age-related trends in ocular findings within these pediatric SCD cohorts.
Main Methods:
- Ophthalmological examinations were conducted on children aged 5-7.5 years diagnosed with SS disease or SC disease.
- Retinal vessel abnormalities, including arteriolar sheathing, peripheral closure, and retinal patches, were documented and analyzed.
- Data were compared between SS and SC disease groups, and trends with age were assessed.
Main Results:
- Arteriolar sheathing was the most common abnormality (51% in SS, 30% in SC).
- Peripheral arteriolar closure occurred in 24% of SS and 16% of SC patients.
- Retinal patches were observed in 37% of SS and 24% of SC children; prevalence increased with age in both groups.
- Vitreous opacities were common and associated with retinal vessel disease.
Conclusions:
- Retinal changes, though more frequent in SS disease, did not reach statistical significance compared to SC disease in this pediatric cohort.
- Peripheral vascular closure and retinal patches show a significant upward trend with age, highlighting the importance of early and continued monitoring.
- These findings in children contrast with the higher prevalence of proliferative retinopathy seen in adults with SC disease.
Abstract:
Ophthalmological examinations were performed on 59 of the 74 (80%) children with homozygous sickle cell (SS) disease and on 37 of the 54 (69%) children with sickle cell-haemoglobin C (SC) disease, aged 5-7.5 years, within the cohort study of sickle cell disease. Arteriolar sheathing was the commonest retinal vessel abnormality, occurring in 30/59 (51%) SS children and in 11/37 (30%) SC children. Peripheral arteriolar closure was observed in 14 (24%) SS children and in 6 (16%) SC children. Arteriovenous anastomoses were seen in 3 children, but proliferative retinopathy was not identified. Capillary changes often occurred in patients without confluent closure, suggesting that complex remodelling of the capillary bed may precede retinal non-perfusion. Discrete retinal patches similar to schisis cavities resulting intraretinal haemorrhages were found in 22 (37%) SS children and in 9 (24%) SC children, but haemorrhages were observed in only 2 patients (1 SS, 1 SC). Vitreous opacities were common and were generally associated with retinal vessel disease. Retinal changes were consistently more common in children with SS disease, though the differences failed to reach statistical significance. The prevalence of peripheral vascular closure and retinal patches showed a significant upward trend with age. These observations contrast with the greater prevalence of proliferative retinopathy characterising SC disease in adults.