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A screening strategy for the detection of sickle cell retinopathy in pediatric patients
Harmeet S Gill1, Wai-Ching Lam
1Department of Ophthalmology & Vision Sciences, University of Toronto, Toronto, ON, Canada.
Insights
Children with sickle cell disease (SCD) should begin retinopathy screening at age 9 for SC genotype and age 13 for SS/SB-Thalassemia genotypes to prevent vision loss. Regular eye exams are crucial for early detection and management.
Area of Science:
- Ophthalmology
- Hematology
- Pediatrics
Background:
- Routine screening for retinopathy is essential in children with sickle cell hemoglobinopathy to prevent vision-threatening complications.
- Understanding the prevalence and age of onset of retinopathy guides effective screening strategies.
Purpose of the Study:
- To determine the prevalence and age of onset of clinically significant retinopathy in pediatric sickle cell patients.
- To recommend an optimized screening strategy for ophthalmologists managing these patients.
Main Methods:
- A retrospective review of 263 pediatric sickle cell patients (up to age 18) was conducted.
- Data analyzed included retinopathy onset, hemoglobin genotype, gender, and systemic manifestations.
Main Results:
- Proliferative retinopathy (PR) was infrequent, observed in 8.2% of SC genotype and 0.6% of SS genotype patients.
- The mean age of PR onset was 13.7 years (SC genotype) and 16 years (SS genotype).
- Gender and systemic manifestations did not predict retinopathy prevalence or onset age.
Conclusions:
- Screening for retinopathy should commence at age 9 for SC genotype and age 13 for SS and SB-Thalassemia genotypes.
- Biennial eye examinations are recommended for normal findings; abnormal results warrant fluorescein angiography and follow-up.
Background:
Children with sickle cell hemoglobinopathy are referred routinely to detect retinopathy and thereby prevent vision-threatening complications. This study aimed to determine the prevalence and age of onset of clinically significant retinopathy in such patients, and to recommend a screening strategy for ophthalmologists.
Methods:
Two hundred sixty-three pediatric sickle cell patients to a maximum age of 18 years during the period of observation were reviewed for the onset of retinopathy, considering the influence of hemoglobin genotype, gender, and the presence of systemic manifestations.
Results:
Proliferative retinopathy (PR) was rare. Six cases (8.2%) of PR were seen in the SC genotype, 1 case (0.6%) in the SS genotype, and no cases in the SB-Thalassemia genotype. The age of onset of PR was a mean of 13.7 years (median 13, range 9-18) in the SC genotype and 16 years in the SS genotype. Neither gender nor the presence of systemic manifestations was predictive for the prevalence or age of onset of retinopathy.
Interpretation:
Screening for retinopathy may begin at age 9 years for SC genotype patients and at age 13 years for SS and SB-Thalassemia genotype patients. Serial examinations may be done biennially for eyes with normal findings. Patients with an abnormal examination should undergo fluorescein angiography and be followed as necessary.
