Introduction: To evaluate the incidence of recurrent intracranial bleeding and mortality in patients with hemorrhagic Moyamoya disease(MMD), and to identify risk factors and the time course of these events.
Methods: A total of 128 patients with complete follow-up treated conservatively were included.The occurrence of recurrent stroke over long-term follow-up was documented. Annual and cumulative incidence rate of rebleeding was generated via Kaplan–Meier survival analysis, and risk factors for rebleeding were analyzed through Cox proportional hazards regression models.
Results: The median follow-up time was 10.1 (range:1–27) years. During 1300.7 patient-years,47(36.7%) patients experienced fifty-nine episodes of rebleeding, rendering an average annual incidence of 4.5%. Among them, 9 patients (19.1%) died from rebleeding and 12 patients had serious disability (modified Ranking Scale=3 ). The cumulative risk of rebleeding was 7.8% at 5 years, 22.6% at 10 years, and 35.9% at 15 years. Only 4 (3.1%) patients experienced ischemic stroke, yield an average annual incidence of 0.3%. Decreased of regional cerebral blood flow (rCBF) (HR, 2.23; P=0.03),smoking and drinking history (HR, 2.43; P=0.04) were independent predictors of rebleeding. Rebleeding (HR,10.76; P=0.02) and hypertension (HR,5.29; P=0.02) were associated with increased mortality. Age, types of first bleeding, DSA stage, family history, and coexistent with cerebral aneurysms were not associated with any increased risk of rebleeding .
Conclusions: Rebleeding events were common in patients with hemorrhagic MMD and the risk of rebleeding increased within a very long-term follow-up. Rebleeding was strongly associated with increased mortality. Decreased of rCBF, smoking and drinking history seem to be risk factors for rebleedin
Patient Care: Clinicians should acknowledge that rebleeding events are still common even more than 10 years after the first bleeding
Learning Objectives: To evaluate the natural course of hemorrhagic MMD in China.