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Causes, clinical and imaging characteristics of moyamoya disease in children

Cai-Xia Wang, Qing-Yi Wang, Yi-Ming Zhao, Mei Zhou, Xiu-Xia Chen, Rui-Yun Zhang

Introduction: Moyamoya disease is a progressive, chronic occlusive vascular disease of the circle of Willis arteries leading to the development of collateral circulation to compensate the occlusion.

Purpose: This study aims to investigate the potential causes and clinical and imaging characteristics of Moyamoya Disease (MMD) in children.

Methods: A retrospective analysis was performed on the clinical data of 14 MMD children, in which the potential causes were analysed, and the clinical and imaging characteristics were observed.

Results: The average patient age was 6.15 y (range 21 months-13 y), and the female/male ratio was 1.3:1.0. There were 332 cases of ischemia and 196 hemorrhages. Eight cases had inducing factors including five cases of upper respiratory tract infection. Twelve cases exhibited limb weakness or hemiplegia. Among these patients, eight cases developed these manifestations as initial symptoms. Furthermore, seven cases presented language barriers, two cases had twitch, and two cases had headaches with unclear vision. All 14 patients underwent cranial Magnetic Resonance Imaging (MRI) and the results revealed that twelve cases had cerebral infarction, and two cases had cerebral hemorrhage. Five of 14 patients experienced stenosis or occlusion at the siphon part of the bilateral internal carotid arteries, seven patients had bilateral anterior cerebral artery stenosis or occlusion, 8 were bilateral middle cerebral artery stenosis or occlusion, and 2 were bilateral posterior cerebral artery stenosis or occlusion. All patients smoke-like vessels in the basal ganglia region.

Conclusion: High-risk age of child MMD ranged between 4-10 y old, which mostly manifested as transient cerebral ischemic insults; and inducing factors were mostly upper respiratory tract infections.