Effects of Surgical Revascularization on Outcome of Patients With Pediatric Moyamoya Disease

Background and Purpose We reviewed surgically treated patients with pediatric moyamoya disease and examined whether vasoreconstructive surgeries reduced the risk of recurrent ischemic attacks and changed overall outcomes in terms of the patients’ performance and intellectual status. Methods Sixty-four hemispheric sides in 34 pediatric moyamoya disease patients who received surgical treatment were examined. We performed superficial temporal artery to middle cerebral artery (STA-MCA) bypass and encephalo-duro-arterio-myo-synangiosis (EDAMS) on 48 sides (combined group) and indirect bypass surgery such as EDAMS on 16 sides (indirect group). These 34 patients were observed postoperatively from 1 to 14 years (mean±SD, 6.6±3.8 years) and were examined for the incidence of recurrent ischemic attack. Of the 34 patients, 23 were followed up for >5 postoperative years, and their overall outcomes in terms of their performance and intellectual status were determined. Results Perioperative ischemic events (≤2 weeks after surgery) occurred in 5 surgeries (31%) of the indirect group and in 6 (13%) of the combined group ( P =NS). The incidence of postoperative ischemic events (>2 weeks after surgery) was significantly reduced in the combined group (10%) compared with the indirect group (56%; P <.01). Of the 23 patients observed >5 years, 7 patients (30%) were mentally retarded and regarded as having a fair outcome. Conclusions Combined surgery (STA-MCA bypass with EDAMS) for pediatric moyamoya disease was effective in reducing the risk of postoperative ischemic attacks compared with indirect surgery. Surgical revascularization may be effective in preventing intellectual deterioration and improving overall outcome.