Clinical Features, Surgical Treatment, and Long-Term Outcome in Pediatric Patients with Moyamoya Disease in China

Abstract

Background: There was few detailed demographic and clinical data about Chinese patients with moyamoya disease. Here we describe the clinical features, surgical treatment, and long-term outcome of pediatric patients with moyamoya disease at a single institution in China. Methods: Our cohort included 288 pediatric patients with moyamoya disease. The demographic and clinical characteristics were obtained by retrospective chart review and long-term outcome was evaluated using the stroke status. Univariate and multivariate logistic regression analyses were performed to determine the risk factors for clinical outcome. The risk of subsequent stroke was determined using the Kaplan-Meier method. Results: The median age for the onset of symptoms was 8.0 years. The ratio of female to male patients was 1:1. Familial occurrence of moyamoya disease was 9.4%. The incidence of postoperative complications was 4.2%. Postoperative ischemic events were identified as predictors of unfavorable clinical outcome, while older age of symptom onset was associated with a favorable clinical outcome. The Kaplan-Meier estimate stroke risk was 5% in the first 2 years, and the 5-year-Kaplan-Meier risk of stroke was 9% after surgery for all patients treated with surgical revascularization. Overall, 86% of patients had an independent life with no significant disability. Conclusion: This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good outcomes. Our results indicate that an early diagnosis and active intervention before the establishment of irreversible hemodynamic change are essential to achieve a favorable clinical outcome.

Surgical outcomes following encephaloduroarteriosynangiosis in adult moyamoya disease associated with Type 2 diabetes

“…5,6,11,14,32,34 Preoperative cerebral infarction, younger age at onset, involvement of the PCA, intentional hypotension, high Suzuki stage, and postoperative ischemic events have been observed to correlate with postoperative complications and outcomes. According to the multivariate logistic regression analyses, our study showed that PCA involvement in both groups with no statistically significant differences preoperatively together with the late postoperative stroke was associated with unfavorable clinical outcomes, which was similar to the previous results observed in our center.…”

“…27 Surgical revascularizations such as direct, indirect, or combined anastomosis have been proven to be effective in improving decreased cerebral circulation and clinical signs. 25 Encephaloduroarteriosynangiosis (EDAS) is an indirect revascularization procedure that has been shown to be beneficial for pediatric 6,12,22 and adult populations. 2,5,7 The results of revascularization due to EDAS are associated with some factors, including age, complications, and other unknown factors.…”

Research Progresses in Understanding the Pathophysiology of Moyamoya Disease

“…Children with MMD usually present with ischemic attacks, whereas adults may have either ischemic or hemorrhagic events [13,14] . Other manifestations include migraine-like headaches and movement disorders.…”

Effect of surgery on the long-term functional outcome of moyamoya disease: a meta-analysis

“…We included studies meeting the following criteria: [1] Observational studies describing patients with the diagnosis of MMD according to the diagnosis and treatment guideline (31) [2] Patients that underwent revascularization surgery either by direct bypass, indirect bypass or combined bypass [3] With an exact definition of good functional outcome [4] Recording the number of patients with good outcome or the rate [5] With follow-up time of at least 6 months.…”