Preoperative Risks of Cerebral Infarction in Pediatric Moyamoya Disease

Background and Purpose: In pediatric moyamoya disease, there have been few reports of the risk factors for preoperative cerebral infarction, especially during the waiting period before surgery. The clinical and radiological findings of surgically treated pediatric moyamoya patients were evaluated to analyze the risk factors for cerebral infarction seen from onset to surgery. Methods: Between August 2003 and September 2019, 120 hemispheres of 71 patients under 18 years of age with moyamoya disease were surgically treated by direct and indirect bypass procedures. The mean age of all surgical hemispheres at diagnosis was 6.7 +/- 3.9 years (6 months-17 years). The potential risk factors for preoperative infarction were examined statistically. Results: Multivariate logistic regression analysis showed that risk factors for infarction at the time of diagnosis were age at diagnosis (odds ratio [OR], 0.68 [95% CI, 0.57-0.82]; P<0.0001) and the magnetic resonance angiography (MRA) score (OR, 2.29 [95% CI, 1.40-3.75]; P=0.001). Univariate analysis showed that risk factors for infarction while waiting for surgery were age at diagnosis (OR, 0.61 [95% CI, 0.46-0.80]; P<0.0001), the MRA score (OR, 1.75 [95% CI, 1.26-2.41]; P=0.0003), and onset of infarction (OR, 40.4 [95% CI, 5.08-322.3]; P<0.0001). Multiple comparisons showed that patients under 4 years of age were at a significantly high risk of infarction at the time of diagnosis and while waiting for surgery. Time from diagnosis to surgery of >2 months was a significant risk factor for infarction while waiting for surgery in patients under 6 years of age. Conclusions: Young age at diagnosis and a high MRA score may be associated with rapid disease progression and result in preoperative infarction. We recommend that surgery be performed within 2 months of diagnosis for the patients under 4 years of age with a high MRA score (>5) and cerebral infarction. Further study is needed to define the optimal timing of surgery.