Revascularization Is Associated With a Reduced Stroke Risk in Patients With Sickle Cell–Associated Moyamoya Syndrome

Abstract

BACKGROUND: Moyamoya syndrome refers to a progressive stenosis of the internal carotid arteries and can be associated with sickle cell disease. These codiagnoses result in severe risk for stroke, even in patients on optimal medical management. Surgical revascularization has been shown to be safe in small case series. OBJECTIVE: To evaluate the efficacy of revascularization with direct comparison to a medically managed control group within a single institution. METHODS: A retrospective cohort study of medically managed vs surgically revascularized patients with moyamoya syndrome and sickle cell disease was conducted. Demographic data and outcomes including the number of prediagnosis, postdiagnosis, and postrevascularization strokes were collected. Risk factors for stroke were identified using a binary logistic regression model, and stroke rates and mortality between groups were compared. RESULTS: Of the 29 identified patients, 66% were medically managed and 34% underwent surgical revascularization (50% direct and 50% indirect). Calculated stroke rates were 1 per 5.37 (medical management), 1 per 3.43 (presurgical revascularization), and 1 per 23.14 patient-years (postsurgical revascularization). There was 1 surgical complication with no associated permanent deficits. No risk factors for stroke after time of diagnosis were found to be significant. CONCLUSION:The results of this study demonstrate that revascularization is associated with a significant reduction in stroke risk, both relative to prerevascularization rates and compared with medical management. According to these findings, surgical revascularization offers a safe and durable preventative therapy for stroke and should be pursued aggressively in this patient population.

Hematologic Disorders and the Nervous System

“…However, patients with SCD may also develop moyamoya syndrome due to progressive vasoocclusive disease of the intracranial arteries. This may lead to further risk of ischemic or hemorrhagic strokes, so prophylactic revascularization procedures can be considered 9 . Close collaboration and follow-up with hematology are important to prevent future vasoocclusive disease.…”

“…This may lead to further risk of ischemic or hemorrhagic strokes, so prophylactic revascularization procedures can be considered. 9 Close collaboration and follow-up with hematology are important to prevent future vasoocclusive disease. stroke and other cerebrovascular complications in the setting of systemic illness.…”

“…Essential thrombocythemia (ET) is another myeloproliferative disorder associated with JAK2 mutation that leads to clonal expansion of megakaryocytes in the bone marrow and thrombocytosis (>450 × 10 9 /L). Similar to other myeloproliferative disorders, ET may be complicated by intravascular thrombus in 10% to 20% of cases, contributing to higher morbidity and mortality 21,27 .…”