Pediatric sickle cell retinopathy: correlation with clinical factors

Sickle cell disease (SCD) occurs in 1 of every 500 African American births and 1 of every 36,000 Hispanic American births. Of children with SCD, 16.7% to 96.3% develop sickle retinopathy (SR). This study was designed to determine whether certain factors are associated with SR and whether SR is correlated with a greater incidence of other SCD manifestations.

A retrospective analysis was performed of 258 children with SCD seen in the ophthalmology clinic at a large urban children's hospital. Of these, 54 children with SR were matched for age and sickle variant with 54 children with normal examinations. Data extracted included demographics, type of retinopathy, presence of glucose-6-phosphate dehydrogenase (G6PD) deficiency, and history of acute chest syndrome, transfusions, pulmonary hypertension, renal disease, cerebrovascular accident, aplastic crisis, splenic sequestration, priapism, osteonecrosis, gallstones, pneumonia, leg ulcers, vaso-occlusive pain crises, and death.

Of the children with SR, 11 (20.3%) had active proliferative disease, 32 (56.1%) had hemoglobin SS, 18 (31.6%) had hemoglobin SC, and 4 (7.0%) had hemoglobin S-beta thalassemia. Several factors were correlated with retinopathy: pain crisis (odds ratio [OR], 5.00; p=0.011), male sex (OR, 4.20, p=0.004), and splenic sequestration (OR, 4.00; p=0.013). G6PD deficiency was more common in patients with retinopathy, although this was not statistically significant (OR, 4.20; p=0.054). No other factors, including frequency of pain crisis, were statistically significant.

Patients with pain crisis and splenic sequestration should be considered for early ophthalmic evaluation. Those with G6PD deficiency may also deserve early screening. By identifying patients at high risk for SR, we can refine screening protocols to safeguard patients from vision loss.