Ocular findings in sickle cell patients on Curaçao

We examined about half the estimated number (based on gene frequency) of sickle cell patients on the island of Curaçao. This study may represent one of the most comprehensive and a selective cross-sectional surveys available. Studying 81 Hb SS and 97 Hb SC patients, we were able to confirm the correlation between iris atrophy and proliferative sickle retinopathy (PSR), the correlation between Hb SC and proliferative sickle retinopathy (PSR) and the major role of PSR in blindness in sickle cell patients. In Hb SC patients over the age of 18 PSR occurred in 50%, leading to vitreous hemorrhage in 18% and to retinal detachment in 8%. We found a frequency of blindness in one eye (fingers counting or less) of 4% in the entire sickle population, and 6% among Hb SC patients. Bilateral blindness was rare: one Hb SS patient, due to cortical blindness. The relatively frequent occurrence of severe complications in a narrowly defined population should stimulate our efforts to prevent these from occurring.