Spontaneous Remodeling of the Peripheral Retinal Vasculature in Sickling Disorders

Risk Factors for Visual Impairment in Patients with Sickle Cell Disease in London

“…Although retinopathy in patients with SCD was first noted in 1956 (9), it was Morton Goldberg's comprehensive work in the early 1970s that helped elucidate the pathogenesis of the condition as well as outline important prognostic factors. After suggesting a classification system for PSR that now bears his name (4), Goldberg used periodic photography and fluorescein angiography to show evolution of peripheral retinal vasculature over time (6). In a study of 45 patients, spontaneous regression of retinal sea fans was demonstrated in 9 patients (8 HbSC, 1 HbSthal), and this was thought to be secondary to autoinfarction (7).…”

“…Much of our understanding of the eye in SCD originates from landmark studies performed by Morton Goldberg and colleagues in the early 1970s (1, 4-5-6-7-8). These studies showed PSR to be considerably more prevalent in HbSC than HbSS patients, with visual loss being significantly more common in the former as a consequence.…”

Sickle cell retinopathy in Jamaican children: further observations from a cohort study.

“…In the majority of children the peripheral vasculature was remarkably stable, with little evidence of the remodelling reported in adults. 16 This hypothesis of a dynamically changing pattern of transient occlusion and reperfusion may explain the relatively slow development of PSR. Only one child, aged 8 …”

Retroequatorial Red Retinal Lesions in Sickle Cell Anemia

“…Whether the fading of the retinal lesions which we observed in these 2 patients with sickle cell disease represents their natural evolution [Condon and Serjeant, 1975;Galinos et al, 1975] or was accelerated by the arteriolar vasodilator treatment cannot be conclusively answered by these 2 case reports. However, it is noteworthy that both of our patients had elevated mid-range red cell den sity values (R60), which have been shown in sickle cell patients to be significantly asso ciated with the severity of the conjunctival 'comma' sign [Roy etal., 1985].…”