Sickle Cell Disease in Children: Accuracy of Imaging Transcranial Doppler Ultrasonography in Detection of Intracranial Arterial Stenosis

Abstract

This study aimed to determine the accuracy of imaging transcranial Doppler sonography in detection of intracranial arterial stenosis in children with sickle cell disease using three-dimensional MR angiography as a reference standard. Sixty-one children (mean age 102±39 months, 30 males), who had no history of overt stroke, and were classified as at lowest risk of stroke by mean flow velocity criterion <170 cm/s, underwent conventional and imaging transcranial Doppler ultrasonographic examinations. We employed the area under the receiver operating characteristic curve (AUC) to determine the accuracy of flow velocity measurements obtained with imaging ultrasonography with and without correction for the angle of insonation as well as with conventional ultrasonography. We also established the most efficacious velocity thresholds for detection of the stenosis. We found ten intracranial stenoses in six patients on MR angiography, but we calculated AUC only for detection of stenosis (n=6) of the left intracranial internal carotid artery. The accuracy of flow velocity with angle correction was lower than the accuracy of velocity without angle correction (AUC=0.73, 95% CI, 0.53-0.93 versus AUC=0.87, 95% CI, 0.74-1.00; p=0.017). The accuracy of flow velocity obtained with conventional ultrasonography (AUC=0.82, 95% CI, 0.67-0.97) was not different from the accuracy of flow velocities obtained with imaging ultrasonography. We found that the threshold of 165 cm/s of mean velocity without angle correction is associated with highest efficiency for imaging (92%) and conventional ultrasonography (90%). Velocity measurements without angle-correction provide good accuracy in detection of stenosis of the terminal internal carotid artery, whereas angle-corrected velocities have lower accuracy.