Assessment of blood supply of the external carotid artery in moyamoya disease using super-selective pseudo-continuous arterial spin labeling technique

Objectives To evaluate the diagnostic accuracy of super-selective pseudo-continuous arterial spin labeling (ss-pCASL) at depicting external carotid artery (ECA) perfusion territory in moyamoya disease (MMD). Methods In total, 103 patients with MMD who underwent both ss-pCASL and digital subtraction angiography (DSA, the reference standard) were included. There were 3, 184, and 19 normal, preoperative, and postoperative MMD hemispheres, respectively. The ss-pCASL results were interpreted by two different visual inspection criteria: presence or absence and definite or indefinite ECA perfusion territory. The performance of ss-pCASL at depiction of ECA perfusion territory was compared to that of DSA. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were calculated. The κ statistic was used to assess intermodality and inter-reader agreement. Results When interpreted as presence or absence, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of ss-pCASL for depicting ECA perfusion territory were 78.3 %, 79.6 %, 92.5 %, 53.4 %, and 78.6 %, respectively, and the intermodality and inter-reader agreement were κ = 0.49 (CI: 0.43 – 0.55, p < 0.01) and 0.71 (CI: 0.66 – 0.76, p < 0.01), respectively. When interpreted as definite or indefinite, the respective values were 61.1%, 100%, 100%, 44.5%, 70.4%, κ = 0.42 (CI: 0.37 – 0.47, p < 0.01), and 0.90 (CI: 0.87 – 0.93, p < 0.01). Conclusion ss-pCASL has substantial sensitivity and specificity compared with DSA for depicting the presence versus absence of ECA perfusion territory in MMD. As a noninvasive method in which no ion radiation or contrast medium is needed, ss-pCASL may potentially reduce the need for repeated DSA examination. Key Points • Super-selective pseudo-continuous arterial spin labeling (ss-pCASL) is a noninvasive vessel-selective MR technique to demonstrate perfusion territory of a single cerebral artery. • Compared with digital subtraction angiography, ss-pCASL has substantial sensitivity and specificity for depicting the perfusion territory of the external carotid artery in brain parenchyma in moyamoya disease. • ss-pCASL may potentially reduce the need for repeated DSA examination .