Purpose:
Moyamoya disease and syndrome represent rare entities characterized by progressive stenosis and/or occlusion of the intracranial blood vessels. We present our series of patients with moyamoya disease and syndrome stratified by underlying disease and analyze differences in presentation and outcome following surgical revascularization.
Methods:
This was an Institutional Review Board (IRB) approved, retrospective review of all patients surgically revascularized by the senior author (SNM) while at Children’s National Hospital in Washington, DC. Demographic data, presenting symptoms and severity, surgical details, and functional and radiographic outcomes were obtained and analyzed for differences amongst the underlying cohorts of moyamoya disease and syndrome as well as by unilateral or bilateral disease and index or non-index surgeries.
Results:
Twenty-two patients were identified with the following underlying diseases: six with idiopathic moyamoya disease, six with sickle cell anemia, five with trisomy 21, and five with neurofibromatosis type 1. Thirty hemispheres were revascularized with a significantly reduced rate of stroke from 3.19 strokes/year (SD = 3.10) to 0.13 strokes/year (SD = 0.25), p = 0.03. When analyzed by underlying cause of moyamoya syndrome, patients with neurofibromatosis type 1 were found to be significantly less likely than the other subtypes of moyamoya syndrome to have had either a clinical stroke (0.0% vs 100.0% [sickle cell], 60.0% [trisomy 21], or 83.3% [moyamoya disease], p < 0.01) or radiographic stroke (0.0% vs 100.0% [sickle cell], 60.0% [trisomy 21], or 83.3% [moyamoya disease], p < 0.01) at time of presentation. Additionally, patients with bilateral disease demonstrated no difference in final functional outcome (mRS 0.73 (SD = 1.33) vs. 1.29 (SD = 1.60), p = 0.63), but second, contralateral surgeries were associated with an increased rate of temporary peri-operative ischemic events when compared to index procedures (50.0% vs 9.1%, p = 0.03).
Conclusion:
Surgical revascularization decreases stroke risk for pediatric patients with different forms of moyamoya disease and moyamoya syndrome. In this study, all but one patient underwent pial synangiosis for indirect revascularization.