Encephalo-duro-myo-synangiosis for the treatment of children with moyamoya disease: long-term angiography and clinical outcome

Objective To investigate the long-term angiography and clinical outcome of encephalo-duro-myo-synangiosis (EDMS) for the treatment of children with moyamoya disease. Methods The clinical and imaging data of before and after procedure in children with moyamoya disease treated with EDMS were analyzed retrospectively. Results A total of 21 children with moyamoya disease were enrolled, including 13 females and 8 males, aged 4 to 16 years. The initial symptom: transient cerebral ischemic attack in 15 cases, ischemic stroke in 4 cases, and hemorrhagic stroke in 2 cases. Matsushima clinical classification: type Ⅰ in 8 cases, type Ⅱ in 7 cases, type Ⅳ in 3 cases, type Ⅴ in 1 case, and type Ⅵ in 2 cases. Suzuki stage: stage Ⅱ in 4 cases, stage Ⅲ in 11 cases, stage Ⅳ in 5 cases, and stage Ⅴ in 1 case. Ten children underwent bilateral operation and 11 underwent unilateral operation (a total of 31 sides). They were followed up for 13 to 91 months (mean 39.8 months). Three children had transient ischemic attack, 2 had cerebral infarction, 7 had facial edema, and none of them died during the perioperative period. The clinical symptoms were improved significantly in 14 sides (45.2%), good in 13 sides (41.9%), and general in 4 sides (12.9%) 1 year after operation. The proportion of children with modified Rankin Scale (mRS) score 0-2 after operation was significantly higher than that before procedure (95.2% vs. 71.4%; χ2=4.29, P=0.041). The middle meningeal artery and deep temporal artery participated in the blood supply of cerebral cortex in different degrees were observed by cerebral angiography again for 31 sides, excellent in 25 (80.6%) and fair in 6 (19.4%). Conclusions The long-term angiography and clinical outcome in children with moyamoya disease treated with EDMS is good. Key words: Moyamoya Disease; Cerebral Revascularization; Cerebral Angiography; Treatment Outcome; Child