Cerebral Vasculopathy in Children With Sickle Cell Disease in an Amazonian Population

Abstract

Introduction: Sickle cell disease is the most prevalent hereditary disease in the country. The aim of this study was to use transcranial Doppler as a screening method for identifying cerebral vasculopathy in children with sickle cell disease. Methods: An epidemiologic, descriptive, and cross-sectional study was conducted. Patients aged 2-16 years with sickle cell disease and followed at a neurology referral service between January 2014 and March 2020 underwent transcranial Doppler and complementary examinations to screen for cerebral vasculopathy. Results: Screening and confirmatory examinations diagnosed 14 of 164 patients (8.5%) with cerebral vasculopathy. Regarding stroke risk, as measured by cerebral blood flow velocity, 2 of 14 patients (14.2%) were classified as conditional risk (170-199 cm/s) and 12 of 14 (85.7%) as high risk of stroke. Conclusion: Complementary examinations should be performed in all patients with changes on transcranial Doppler to confirm cerebral vasculopathy. Further studies, particularly genetic, are needed to better understand the relationship between sickle cell disease and cerebral vasculopathy.