Differences in Intracranial Artery and Brain Impairment Between Pediatric and Adult Moyamoya Disease: A Comparative MRI Study.

Abstract

Background: Moyamoya disease (MMD) exhibits incidence peaks in children and adults, but the age-related differences in MR imaging features remain unclear.

Purpose: To determine the differences in intracranial artery and brain impairment between pediatric and adult patients with MMD using MR imaging.

Study type: Retrospective.

Population: Four hundred and three patients (median age, 42 years [IQR, 29-51 years]; 268 adults) diagnosed with MMD by digital subtraction angiography (DSA), with routine clinical brain MRI and high-resolution intracranial vessel wall imaging within 1 week of DSA.

Field strength/sequence: Pre- and post-contrast 3D T1 SPACE and routine brain MRI sequences including T1-weighted, T2-weighted, FLAIR, diffusion weighted imaging, and 3D time-of-flight at 3.0 T.

Assessment: Three neuroradiologists (10, 6, and 11 years of neurovascular imaging experience) assessed vessel wall enhancement grades and patterns, basal ganglia and centrum semiovale perivascular spaces, white matter hyperintensities, lacunes, infarction, hemorrhage, and intracranial arterial luminal stenosis.

Statistical tests: Student's t-test, Mann-Whitney U test, Chi-square test, or Fisher's exact test, and logistic regression analysis. A two-sided p value < 0.05 was considered statistically significant.

Results: Adult patients were significantly more likely to have bilateral intracranial internal carotid artery stenosis (90.7% vs. 81.5%), Grade 2 enhancement in the posterior cerebral artery (9.0% vs. 2.2%), and significantly higher risk of intracranial hemorrhage (odds ratio (OR) = 5.216, 95% confidence interval (CI): 2.312-11.767), periventricular WMHs (OR = 2.634, 95% CI: 1.263-5.494), and lacunes (OR = 2.012, 95% CI: 1.179-3.432) compared to pediatric patients. In contrast, pediatric patients were significantly more prone to bilateral anterior cerebral artery (ACA) stenosis and vessel wall enhancement, and had significantly higher prevalence of Grade 1 enhancement at the origins of the middle cerebral artery and ACA, as well as significantly higher odds of PVSs in the centrum semiovale (OR for adults = 0.447, 95% CI: 0.210-0.949) compared to adult patients.

Data conclusion: The intracranial arterial and brain impairments assessed by MR imaging significantly differed between adult and pediatric patients with MMD.

Evidence level: 3.

Technical efficacy: Stage 5.