Contrast-Enhanced Color-Coded Doppler Sonography in Moyamoya Disease: A Retrospective Study

The purpose of this study was to validate the feasibility of contrast-enhanced transcranial Doppler sonography (CE-TCCD) in the diagnosis of Moyamoya disease (MMD). CE-TCCD data on patients with MMD were analyzed. The CE-TCCD data were classified qualitatively into four patterns by two independent investigators: normal vascular color Doppler signal (pattern 1), augmented color Doppler signal with identifiable vascular structure (pattern 2), confluent color Doppler signal filling more than two-thirds of the display frame without identifiable vascular structure (pattern 3) and confluent color Doppler signal filling full display (pattern 4). To investigate the validity, we compared the CE-TCCD data with traditional transcranial Doppler data and Suzuki grades on cerebral angiography. A total of 32 CE-TCCD studies from 16 MMD patients (male 37.5%, median age 48) were included in this study. The CE-TCCD findings were distributed across patterns 1 (n = 3), 2 (n = 12), 3 (n = 10) and 4 (n = 7) and were correlated with the Suzuki grades (p < 0.005) and hemodynamic parameters. Inter-rater reliability was promising (Cronbach α = 0.883). The CE-TCCD test provides distinctive patterns in MMD, according to their stage of progression. CE-TCCD patterns seem to be a reliable and valid means for the evaluation of MMD.