Clinical outcome after 450 revascularization procedures for moyamoya disease

Clinical article

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Object

Moyamoya disease (MMD) is a rare cerebrovascular disease mainly described in the Asian literature. To address a lack of data on clinical characteristics and long-term outcomes in the treatment of MMD in North America, the authors analyzed their experience at Stanford University Medical Center. They report on a consecutive series of patients treated for MMD and detail their demographics, clinical characteristics, and long-term surgical outcomes.

Methods

Data obtained in consecutive series of 329 patients with MMD treated microsurgically by the senior author (G.K.S.) between 1991 and 2008 were analyzed. Demographic, clinical, and surgical data were prospectively gathered and neurological outcomes assessed in postoperative follow-up using the modified Rankin Scale. Association of demographic, clinical, and surgical data with postoperative outcome was assessed by chi-square, uni- and multivariate logistic regression, and Kaplan-Meier survival analyses.

Results

The authors treated a total of 233 adult patients undergoing 389 procedures (mean age 39.5 years) and 96 pediatric patients undergoing 168 procedures (mean age 10.1 years). Direct revascularization technique was used in 95.1% of adults and 76.2% of pediatric patients. In 264 patients undergoing 450 procedures (mean follow-up 4.9 years), the surgical morbidity rate was 3.5% and the mortality rate was 0.7% per treated hemisphere. The cumulative 5-year risk of perioperative or subsequent stroke or death was 5.5%. Of the 171 patients presenting with a transient ischemic attack, 91.8% were free of transient ischemic attacks at 1 year or later. Overall, there was a significant improvement in quality of life in the cohort as measured using the modified Rankin Scale (p < 0.0001).

Conclusions

Revascularization surgery in patients with MMD carries a low risk, is effective at preventing future ischemic events, and improves quality of life. Patients in whom symptomatic MMD is diagnosed should be offered revascularization surgery.

Abbreviations used in this paper: EEG = electroencephalography; MCA = middle cerebral artery; MELAS = myopathy, encephalopathy, lactic acidosis, and stroke; MMD = moyamoya disease; mRS = modified Rankin Scale; STA = superficial temporal artery; TIA = transient ischemic attack.

Article Information

Address correspondence to: Gary K. Steinberg, M.D., Ph.D., Department of Neurosurgery, Stanford University School of Medicine, 300 Pasteur Drive, R281 Stanford, California 94305-5487. email: gsteinberg@stanford.edu.

Please include this information when citing this paper: published online May 22, 2009; DOI: 10.3171/2009.4.JNS081649.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    A: Bimodal distribution of age at initial presentation, with peaks in the 10–19 and 30–39 bins. B: Sex breakdown of each bin: consistently two-thirds female and one-third male in each bin except for the 0–9 bin where the ratio is nearly 1:1. C and D: Ethnic distribution (C) and percentage of adult and pediatric patients at presentation in the ethnic subgroups (D).

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    Upper and Lower: Bar graphs showing presenting symptoms in 96 pediatric (upper) and 233 adult (lower) patients consisting of stroke, TIAs, intracerebral hemorrhage (ICH), seizures, and headaches. Adults presented significantly more often with intracerebral hemorrhage than pediatric patients (p < 0.05). A = Asian; AA = African-American; C = Caucasian; H = Hispanic.

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    Bar graphs demonstrating presurgical (A) and postsurgical (B) mRS scores obtained in 264 patients with a minimum of 6 months of follow-up. C: Overall 67% of the patients improved by 1–2 points on the mRS scale (change in mRS score of −1 or −2), whereas 23% remained unchanged (no change in mRS = 0). Status in only a few patients worsened after surgery (change in mRS score of 1–4). Values to the left of the dashed line represent no change or improvement in the mRS score.

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    Kaplan-Meier survival curve for any stroke or hemorrhage. The overall 5-year risk of any stroke or hemorrhage was 5.5%.

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