Ethnic Differences in Childhood Moyamoya Presentation and Evolution

Abstract

Background Emerging evidence suggests that moyamoya natural history differs based on ethnic background and underlying cause. We sought to compare clinical and radiographic presentation and evolution of moyamoya vasculopathy based on ethnic background. Methods We conducted a single-institution retrospective review a cohort of children with moyamoya followed from 2003 to 2019. Clinical and radiographic characteristics at diagnosis were obtained. Clinical evolution and radiographic progression were assessed on subsequent clinical visits and neuroimaging. We compared moyamoya subtypes (moyamoya disease (MMD) vs moyamoya syndrome (MMS) and MMD vs neurofibromatosis type 1 (MMS-NF1) vs sickle cell disease (MMS-SCD)), clinical and radiographic features at presentation, time to clinical event recurrence and radiographic progression and PSOM at last follow-up based on ethnic background. Comparisons were conducted using t-test, chi-square test, logistic and Cox regressions (p Results One hundred and seven patients were identified, of whom 46 had MMD and 61 MMS (22 MMS-NF1, 21 MMSSCD, 18 others). Thirty percent were Caucasian, 19.63% were Black, 17.76% were East Asian and 15% were non-East Asian. The ethnic background of the remainder was unknown. Ethnic distribution based on moyamoya subtype was significant (p Conclusion There are ethnic differences at presentation and follow-up in childhood moyamoya. East Asian patients are more likely to present with TIA and have TIA recurrence and Black patients are less likely to have an ivy sign at presentation. These differences are most likely due to different underlying diagnosis.