Transcranial color-coded duplex sonography reliably identifies intracranial vasculopathy in adults patients with sickle cell disease.

In order to prevent stroke, screening for disease-related intracranial vasculopathy using Doppler is recommended in sickle-cell disease (SCD) children. How to screen such vasculopathy in adults remains largely unknown. The objective of this study was to assess whether Transcranial color-coded duplex sonography (TCCD) is sensitive and specific to identify SCD adult patients with vasculopathy, compared with magnetic resonance angiography (MRA). SCD adults followed in referral centers at high risk of vasculopathy were included in this study. TCCD examination and 3D time-of-flight MRA were performed on the same day. On MRA, vasculopathy was defined by the presence of at least one ≥50% arterial stenosis. On TCCD, vasculopathy was defined by a time-averaged mean of the maximum velocity (TAMx) stenotic/prestenotic ratio ≥ 3, an occlusion, or a Moya-Moya pattern. Vasculopathy was also considered as present when TAMx ratio could not be calculated because of the presence of severe cervical lesions. Among 80 included patients, quality of MRA was insufficient in 3 patients. Among the 38 patients with vasculopathy on MRA, 37 had a vasculopathy according to TCCD criteria: TAMx ratio ≥3 or intracranial occlusion in 33 and cervical lesion in 4. Moya-Moya pattern was identified with TCCD in all 17 patients with Moya-Moya on MRA. Sensitivity and specificity of TCCD to identify patients with ≥50% vasculopathy on MRA were (n=37/38) 97% and (n=28/34) 82%, respectively. Positive and negative predictive values were (n=37/43) 86% and (n=28/29) 97%, respectively. TCCD may be used to identify SCD adult patients with vasculopathy. This article is protected by copyright. All rights reserved.