Ocular manifestations in SCD patients: role of fundus fluorescein angiography in the detection of sickle retinopathy

Background Sickle cell retinopathy comprises a constellation of abnormal signs in the retinas of patients with sickle cell disease (SCD). The aim was to detect ophthalmological complications in patients with SCD, particularly macular microvascular alteration, using fundus fluorescein angiography (FFA), and to assess the role of potentially contributory clinicopathological factors including transcranial Doppler, genotypes, hydroxyurea, transfusion therapy, and finally, iron overload state on the development of sickle eye. Patients and methods This cross-sectional study included 30 patients with SCD randomly recruited from the Paediatric Haematology Clinic of Ain Shams University Children's Hospital, Cairo, Egypt. Complete blood count, transcranial Doppler, and FFA were done. Results Of 30 patients, five had abnormal/conditional transcranial results. The FFA finding was normal in 60 eyes (all 30 SCD patients), and only one patient had abnormal visual acuity. This was a 29-year-old girl who was on simple blood transfusion and hydroxyurea treatment for recurrent cerebral strokes and vaso-occlusive crises, yet her FFA finding was normal. Conclusion The authors could not demonstrate any retinal microvascular alternations using FFA among patients with SCD. The authors related the results to their young age and the fact that all were on hydroxyurea therapy with fair compliance. Further studies using a large sample size are warranted to illustrate the utility of FFA as a tool for early detection of sickle retinopathy. Egypt J Haematol 2020 45:142-147 (C) 2021 The Egyptian Journal of Haematology