• Cunxin Tan, Ran Duan, Xun Ye, Dong Zhang, and Rong Wang.
• Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; China National Research Center for Neurological Diseases, Beijing, China.
• World Neurosurg. 2016 Dec 1; 96: 222-229.

Background And PurposeMoyamoya disease (MMD) is a chronic cerebrovascular disorder with little known etiology. We aim to propose a new classification system for MMD from the perspective of embryology.MethodsMMD patients' digital subtraction angiograms were retrospectively analyzed. Every angiogram was analyzed to find the abnormal vessels and from which part of the posterior cerebral artery (PCA) the lesions begin.ResultsIn 262 MMD cases, 32 pediatric patients had PCA involvement, of which 17 were male and 15 were female; 68 adults had PCA involvement, of which 33 were male and 35 were female. The initially affected part of the PCA was compared between sexes and between pediatric and adult patients, and the findings are not statistically significant (P = 0.233, P = 0.855, P = 0.343, respectively). However, of the 100 cases with PCA involvement, only 4 had the lesions begin from the first part of the PCA, and all of the 4 cases had the basilar artery lesions. All the other 96 cases had the lesions begin from the second part of the PCA or from the posterior communication artery, which is derived from the caudal ramus of the primitive intracarotid artery, leaving the first part of the PCA and basilar artery excluded from affection.ConclusionMMD should be classified into primitive intracarotid artery system-involved type and primitive vertebral basilar artery system-involved type. The reason that the vertebral basilar artery is so rarely involved in MMD might be because of its late development in the brain.Copyright © 2016 Elsevier Inc. All rights reserved.

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