Natural Course and Clinical Outcomes of Patients With Hemorrhagic Moyamoya Disease

BACKGROUND AND OBJECTIVES: Given the natural history of hemorrhagic moyamoya disease (hMMD) is unclear and research on long-term outcomes following indirect revascularization is limited, this study sought to assess its natural course and the efficacy of indirect revascularization. METHODS: We conducted a retrospective analysis of patients with hMMD admitted to the Chinese PLA General Hospital from April 2002 to March 2022. Clinical characteristics, radiologic findings, rebleeding events, and prognostic outcomes were evaluated. Logistic and Cox regression models identified risk factors for poor outcomes and rebleeding in the natural history. After overlap weighting to balance the encephaloduroarteriosynangiosis (EDAS) and conservative groups, weighted regression models were used to compare outcomes. DISCUSSION: In conclusion, hMMD occurs mainly in adult patients. In the natural history of hMMD, AChA dilation was associated with rebleeding, while female sex, AChA dilation, and bilateral lesions were associated with poor outcomes. This study suggests that EDAS is associated with a reduced incidence of rebleeding and improved outcomes, indicating that EDAS may be an effective treatment for hMMD. However, given the retrospective design, prospective validation is needed.