[Vision-threatening eye manifestations in patients with sickle cell disease on Curaçao]

Prospectively we performed an ophthalmological examination in 81 Hb SS and 97 Hb SC patients on the island of Curaçao, about half the estimated number of patients, based on gene frequency. These patients had not been selected on ophthalmological symptoms. Proliferative retinopathy was present in 2% of the Hb SS patients, and in 50% of the Hb SC patients (71% among those over 18 years old). The frequency of severe visual loss (counting fingers or less) in one eye due to sickle cell disease in Hb SS patients was 1% (one patient, both eyes), and in Hb SC patients 6% (6 patients, 6 eyes). Bilateral blindness was rare: one Hb SS patient had cortical blindness. Visual loss in Hb SC patients (of which four eyes with loss of light perception) was caused by proliferative retinopathy, with its complications like vitreous haemorrhage and retinal detachment. Although the effectiveness of prophylactic laser treatment remains to be established, it would appear prudent to have an eye examination performed regularly in Hb SC patients older than 20 years.