ABSTRACT
Background: Sight-threatening retinopathy in Sickle Cell Disease is thought to be due mainly to vasoocclusion. Yet it is reportedly rarely found in children with Haemoglobin SS; (who most often suffer from vasoocclusion). However; earlier reports included patients with a wide range of clinical severity. Aim: To document ocular pathology in children with Haemoglobin SS with severe clinical disease. Methods: Thirty-seven children with severe clinical disease (at least 3 vaso-occlusive episodes in one year) had detailed ocular examinations over a one-year period. Results: No child (aged 3 to 13 years) had ocular symptoms. Visual acuity was abnormal in one child. Retinal pathology was found only in patients over 8 years. Neovascularization was observed in 3 eyes of 2 patients both of whom had higher than average irreversibly sickled cell counts and haemoglobin levels. Retinal and choroidal infarcts were found in 11 and 2 eyes respectively; sunburst lesions and salmon patch haemorrhages in 5 eyes each. Changes observed over the one-year period in the 32 survivors; were photocoagulation scars in one eye of a child who had undergone laser therapy and resolution of the salmon patch haemorrhages. Conclusion: Despite lack of visual symptoms; young children with haemoglobin SS with severe clinical symptoms can develop sight-threatening retinopathy. The possible role of autoinfarction in the causation of these lesions is discussed. Our study shows that routine yearly ophthalmological examinations are essential for children over 8 years