First Author: M.Ugarte UK
Co Author(s): S. Zogana S. Kotsiopoulou J. Maitland
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Assessment of sickle cell's retinopathy frequency and visual disturbance in patients with Haemoglinopathy SS +SC.
Retrospective analysis of retinal complications associated with sickle cell anaemia in Croydon University Hospital.
The sample was comprised of 112 haemotology patients (47 HbSC, 65 HbSS). Thirty five SC ( 25 women, 10 men, mean age ± SD, 43.2± 8.9 years, range 29-61) and 30 SS (21 women, 9 men, mean age ± SD, 35.8± 10.5years, range 19-60) patients had complete ophthalmology records. Visual acuity was measured with Snellen Chart and retinopathy was evaluated with slit lamp examination and wide-angle fundus photographs.
Best corrected visual acuity was greater than or equal to 6/9 bilaterally in 90% of SS patients and 65.7% of SC patients; and less than or equal to 6/18 in the best eye only in one SC patient and none of SS. Risks factors of visual loss was SC genotype and the stage of retinopathy. Stage 0-2 (non-proliferative retinopathy) was found in 85% of SS and 30% of SC patients, stage 3 (proliferative retinopathy) in 13.3% of SS and 40% of SC patients, stage 4 (vitreous haemorrhage) in 1 eye of SS and 10% eyes of SC patients and finally stage 5 (retinal detachment) in none of SS patients and in 20% eyes of SC patients. Eleven eyes (9 patients) with stage 3 retinopathy had undergone laser panretinal photoagulation and eleven eyes with stage 5 retinopathy (7 patients, 4 males, 3 females, age range 26-55) had had retina detachment surgery ( i.e pars plana vitrectomy with or without gas/oil tamponade). BCVA after vitrectomy was 6/6 (1 eye), 6/9 (3), 6/12 (1), 6/60 (3) and 2/60, hand movements and perception of light in one respetively.
Visual loss is rare in SC and SS patients. The advanced stages of retinopathy is the main cause and they do not always respond well to treatment. The pathogenesis and modifiable risk factors for sickle cell retinopathy are not well understood. Further research is required with the aim to develop strategies for the prevention of retinopathy such as treatment of disregulated zinc homeostasis.