• Wenjun Shen, Bin Xu, Hao Li, Xiaofeng Gao, Yujun Liao, Wei Shi, Rui Zhao, and Yi Zhang.
• Department of Pediatric Neurosurgery, Children's Hospital of Fudan University, Shanghai, China. Electronic address: wenjunshen@fudan.edu.cn.
• World Neurosurg. 2017 Oct 1; 106: 9-16.

ObjectiveTo retrospectively evaluate the midterm therapeutic effect of enlarged encephalo-duro-myo-synangiosis (EDMS) for moyamoya disease (MMD) in young children.MethodsSeventy-seven children diagnosed with MMD by digital subtraction angiography (DSA) or magnetic resonance angiography (MRA) were treated between January 2011 and December 2014 in our center. Their clinical features, imaging, and operative reports were analyzed.ResultsFour patients presented with intracerebral hemorrhage, whereas 73 presented with ischemic symptoms. Revascularization procedures were performed unilaterally on 11 left hemispheres and 9 right hemispheres, and 57 had bilateral surgeries. The average length of surgery was 143 ± 24 minutes, with 28 ± 9 mL of blood loss. The mean period of follow-up was 28.43 ± 15.31 months. Cerebral blood flow increased 3 months after the operations in the previously affected regions. Collateralization from the deep temporal artery, superficial temporal artery, and the middle meningeal artery was found by DSA or MRA. In cases where single-photon emission computed tomography was obtained, it demonstrated better perfusion postoperatively. The ischemic symptoms were relieved in 118 (88.06%) hemispheres, and remained stable in 5 (3.73%) hemispheres. There were 12 radiographic cerebral infarctions (8.96%) within 1 month postoperatively.ConclusionsEnlarged EDMS is safe and effective for MMD in young children. Extensive and multilayered revascularization could significantly preserve neurologic function. The long-term effect on posterior circulation disease development needs further investigation.Copyright © 2017 Elsevier Inc. All rights reserved.

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