A screening strategy for the detection of sickle cell retinopathy in pediatric patients.

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.