Differing Surgical Outcomes in a Multiethnic Cohort Suggest Racial Phenotypes in Moyamoya Disease

Background: Variable Moyamoya disease (MMD) genotypes and phenotypes between different races have been suggested previously. This study investigates differences in surgical complications and response to revascularization among Asian patients with MMD compared with other ethnicities in North America. Methods: From a database of 185 patients with moyamoya presenting to our institution between 1994 and 2015, 85 patients with MMD underwent surgery and constituted the study cohort. Baseline characteristics before surgery, procedure-related complications, length of hospital stay, and outcome variables including stroke and functional outcome were compared between Asian and non-Asian patients. Kaplan−Meier survival analyses were used to compare time to ipsilateral stroke and any cerebrovascular event after bypass. Results: Our surgical cohort consisted of 27% (23/85) Asian and 73% (62/85) non-Asian patients with MMD with a bimodal age distribution. Among the subset of patients who presented with stroke (n = 55), hemorrhage was significantly more common among Asian patients (P = 0.007). In 120 revascularization procedures, per-operative complication rates were greater among Asian patients while controlling for age, type of surgery, and stroke history (odds ratio 2.94; 95% confidence interval 1.16–7.48; P = 0.02). The mean follow-up time after surgery was 4.57 years. Ipsilateral cerebrovascular event rates were 4.77 per 100 person-years in non-Asian patients and 6.51 per 100 person-years in Asians (P = 0.66). Unfavorable modified Rankin Scale scores (>2) were found in 22% of Asian patients and 8% of non-Asian patients on last follow-up (P = 0.13). Conclusions: Asian patients with MMD may be more susceptible to surgical complications and may differ from other races in their response to revascularization. Further long-term prospective studies are needed to investigate these findings.