Introduction: The natural history of hemorrhagic Moyamoya disease (MMD) is poor due to high rehemorrhage rates reported to be between 32-61%. Post-revascularization these rates have been reported to decrease to 12-17%. We report the clinical outcomes of one of the largest contemporary series of surgically treated hemorrhagic MMD patients.
Methods: All patients treated surgically for hemorrhagic MMD over 23 years were retrospectively identified from a prospective database. Modified Rankin score (mRS) was used to grade clinical status at presentation and assess outcomes with good being defined as mRS = 2. Univariate and multivariate logistic regression analyses was used to determine risk factors for postoperative morbidity.
Results: Eighty-five patients (57 female; 28 male) with a mean age of 38 years (3-64) were identified. 56 had bilateral disease. Of 143 performed revascularization procedures, 127 were direct superficial temporal artery (STA) - middle cerebral artery (MCA) bypasses; 16 were indirect. Mean pre-operative mRS score was 1.4. Over a mean follow-up period of 5.2 years, eight patients rehemorrhaged and all had clinically significant strokes. At the last documented follow-up, mean mRS score improved to 1.2. Older patients, patients with mRS score = 3 at the time of treatment, left sided bleed, and basal ganglia or thalamic bleed were at risk for poorer outcomes (p < 0.05).
Conclusions: Surgical revascularization of hemorrhagic MMD reduced the rehemorrhage rates to 9.4% (1.8%/ yr) compared with much higher historical rates. The reported outcomes support the strategy to perform revascularization procedure in these patients with a preference for direct STA-MCA bypasses. Longer follow-up data among a larger cohort of patients will further elucidate the validity of this strategy.
Patient Care: Our results support the use of direct cerebral vascularization in reducing the rates of intracranial hemorrhage in patients with hemorrhagic MMD. The current results have significant implications for management of this patient cohort and improving their outcomes.
Learning Objectives: The participant should be able to a) demonstrate awareness of the current literature on hemorrhagic MMD; b) learn regarding existing treatment options and c) appreciate the controversies in the management of this disease entity particularly in light of our presented results.