Sickling Hemoglobinopathies

Abnormalities in the fine vasculature of the macular and perimacular regions occurred in 7 of 35 consecutive patients with sickling hemoglobinopathies (4 sickle cell anemia, 2 sickle cell hemoglobin C disease, 1 sickle thalassemia). Microaneurysm-like dots, dark and enlarged segments of terminal arterioles, and hairpin-shaped venular loops were visible ophthalmoscopically. Pathologic avascular zones and changes in the normal architecture of both the foveal avascular zone and the surrounding capillary bed were visible only on fluorescein angiography. Four of the seven patients had some loss of central visual functions. These abnormalities are similar to changes described In the retinal periphery of patients with sickling disorders and can be explained by the same pathogenic mechanisms. They, therefore, represent a clinical disease that we have called sickling maculopathy.