To investigate the clinical features, surgical treatment and curative effect of pediatric moyamoya disease.

A group of 17 pediatric patients with moyamoya disease (aged 3-14 years ) was retrospectively analyzed from June 2017 to June 2020 in Henan Provincial People's Hospital, in regard to clinical data, imaging features, surgical methods and postoperative treatment. Totally 17 cases (26 sides) underwent brain-dural-temporal muscle-vascular fusion (encepho-dural-myo-synangiosis, EDMS). Postoperative follow-up was conducted, with magnetic resonance angiography (MRA) or digital subtraction angiography (DSA) and perfusion imaging (PWI) or arterial spin labeling (ASL), to evaluate the efficacy of long-term cerebral revascularization after surgery. The improved Rankin scale score (mRs) was used to evaluate the neurological functional recovery.

Six months after the operation, MRA or DSA showed that external carotid artery system compensated blood supply to the cerebral cortex on 24 sides, and PWI or ASL showed that cerebral perfusion ischemia was significantly improved. Totally 24 side surgeries (92.3%) improved the symptoms of neurological impairment, while in the other 2 side surgeries (7.7%), the symptoms were stable, with no new-onset symptoms of neurological impairment. Transient epileptic seizures occurred after 3 operations; local scalp necrosis occurred after 1 operation; and no serious complications such as cerebral infarction and cerebral hemorrhage occurred.

EDMS is safe in the treatment of children with moyamoya disease. It can significantly improve cerebral perfusion, and shows a good effect during short-term follow-up.