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Periodic photographic and angiographic surveys of patients with the earliest stages of sickle retinopathy showed a number of fundus findings. In seven cases (sickle cell anemia, four; sickle cell hemoglobin C, three), these findings included: (1) a variety of vascular abnormalities in the equatorial and post-equatorial retina such as segmented dilations of the vessel walls, hairpin-shaped vascular loops, hypertrophic, tortuous A-V anastomoses, intraluminal plugs, closure and loss of capillary bed, and terminal budding of capillaries; and (2) a continuous, spontaneous remodeling of the peripheral retinal vasculature due to successive closures and reopenings of equatorial retinal vessels. A centripetal recession of the peripheral retinal vasculature usually resulted. No correlation between the ophthalmoscopic and the systemic condition of the patients could be made.

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.

One possible explanation for the rarity of ocular herpesvirus infections in newborn infants may be the universal application of Crede prophylaxis. To test this hypothesis, a clinically used silver nitrate preparation was instilled in the eyes of rabbits 20 minutes after an ocular inoculation with a strain of type 1 or a strain of type 2 herpesvirus (HSV). With the strain of type 1 HSV, silver nitrate prevented infection with smaller inocula (3,000 plaqueforming units [PFU]), but not with a larger dose (30,000 PFU). With the strain of herpes type 2 there was not prevention of disease with either concentration of inoculum (1,000 and 10,000 PFU). The practical importance of silver nitrate prophylaxis on herpetic infection in the newborn may be small.