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Updated: Jul 29, 2026
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
[Sickle cell retinopathy in children: Report of 42 cases]
D Saadouli1, S Yahyaoui2, S Ben Issa1
1Service d'ophtalmologie, CHU La Rabta, 1091 Tunis, Tunisie.
Insights
Sickle cell retinopathy is a common complication in children, with low fetal hemoglobin (HbF) levels being a significant risk factor for severe disease progression. Early detection and management are crucial to prevent vision loss.
Area of Science:
- Ophthalmology
- Hematology
- Pediatrics
Background:
- Sickle cell retinopathy is a serious complication of sickle cell disease (SCD).
- Understanding its epidemiological, etiological, and clinical features in children is essential for timely intervention.
- Identifying risk factors for severe retinopathy can guide preventative strategies.
Purpose of the Study:
- To describe the characteristics and clinical course of sickle cell retinopathy in pediatric patients.
- To determine risk factors associated with severe sickle cell retinopathy.
Main Methods:
- Retrospective chart review of children diagnosed with sickle cell retinopathy.
- Classification of patients into Goldberg stages 1-2 (Group 1) and 3-5 (Group 2).
- Logistic regression analysis to identify independent risk factors for severe retinopathy.
Main Results:
- Sickle cell retinopathy occurred in 14.48% of patients.
- Forty-two children (mean age 14 years) with genotypes SS, SC, Sβ, and SO Arab were included.
- A low fetal hemoglobin (HbF) level (<15%) was the only independent risk factor for severe sickle cell retinopathy.
Conclusions:
- Retinopathy is a frequent complication of sickle cell disease, potentially leading to blindness.
- Lower HbF levels are negatively correlated with the severity of sickle cell retinopathy.
Abstract:
We aimed to describe the epidemiological, etiological and clinical features, treatment and clinical course of sickle cell retinopathy in children and to determine the risk factors for serious involvement.
Methods:
This was a retrospective study including all children diagnosed with sickle cell retinopathy. Epidemiological, clinical and therapeutic characteristics, as well as clinical course, were analysed retrospectively by chart review. Two groups were defined: Group 1 (Goldberg stage 1 and 2); Group 2 (Goldberg stage 3, 4 and 5). In order to identify factors independently associated with severe sickle cell retinopathy, we conducted a logistic regression analysis in descending order.
Results:
The frequency of sickle cell retinopathy was 14.48%. Forty-two patients (84 eyes) were included; among them 23 boys and 19 girls, aged 10 to 17 with a mean age of 14±1.98 years. Twenty patients were of genotype SS, 11 patients of genotype SC, 8 Sβ and 3 SO Arab. The three patients in group 2 were all of SS genotype. The majority of patients (32) had an HbF level of less than 15%. All our patients had sickle cell retinopathy distributed as follows: 62% at stage 1; 31% at stage 2; 5% at stage 3 and 2% at stage 4. Multivariate analysis revealed a single risk factor independently linked to severe involvement - an HbF level<15%.
Conclusion:
Retinopathy is a frequent complication of sickle cell disease which may lead to blindness. The HbF level is negatively correlated with severe involvement.
