Monitoring Sickle Cell Disease Using Spectral-Domain Optic Coherence Tomography In Pediatric Patients

Introduction: Sickle cell disease (SCD) affects multiple organs including the eye. Ophthalmologists have long relied on funduscopic exam to detect sickle cell retinopathy (SCR). Regular examinations to diagnose and monitor SCR permit timely initiation of treatment to prevent vision loss. Clinical guidelines recommending yearly retinal exam beginning at age 10 were based on data using conventional exam techniques. Optic coherence tomography (OCT) is a quick, noncontact test that provides images of distinctive retinal cell layers at high resolution. OCT has revolutionized the ability to examine retinal structure and provided new information on retinal damage in SCD (Bonanomi, 2013, Chow 2011). Our analysis using data from 69 patients shows that OCT has a higher detection rate for retinal changes in SCD, offering earlier diagnosis than fundoscopy alone (Jin 2018). In the pre-OCT era, patients with the SC genotype, although exhibiting less severe systemic SCD manifestations, were more frequently diagnosed with SCR and seemed to develop vision-threatening proliferative SCR more often than those with SS. In this study, we investigated the correlation between SCD genotype and retinal damage detected by OCT, using our expanded dataset to 97 patients.