New Insights On The Anatomy And Function Of The Retina In Sickle Cell Disease

Introduction The recent rise of OCT showed that asymptomatic atrophy of the median temporal raphe of the retina (ATR) was a frequent occurrence in sickle cell disease (SCD). However, the prevalence, mechanisms and significance of ATR is not known, especially in the paediatric field. Methods SCD-children prospectively underwent a fundus examination and an OCT of the macula and the temporal retina. Atrophy of the MTR was quantified on thickness maps. Data from brain imaging were collected in SS-type SCD children. Results Eighty-one children were studied; 64.2% exhibited ATR, while 29.2% had peripheral SCD retinopathy. ATR was often limited to areas located temporally from the usual macular map OCT programs, so that it could be missed with these programs. Patients with peripheral retinopathy were more likely to also exhibit ATR (p = 0.02). While peripheral retinopathy was significantly associated with the children age (p = 0.02), ATR was not. Conclusion ATR seems to occur very early in children with SCD and to display most distinct features from the well-known peripheral SCD retinopathy.