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High prevalence of pro-thrombotic conditions in adult patients with moyamoya disease and moyamoya syndrome: a single center study

  • Original Article - Vascular Neurosurgery - Other
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Abstract

Background

Moyamoya Disease (MMD) and moyamoya Syndrome (MMS) have been reported to be associated with pro-thrombotic states in some patients. To date, however, such reports have been limited to case reports or small case series. We sought to determine the prevalence of pro-thrombotic states among a large cohort of both MMD and MMS patients.

Methods

We retrospectively reviewed the medical records of all adult patients who were diagnosed with MMD or MMS from our institution. In addition to basic demographic and clinical data, the presence of certain pro-thrombotic conditions was noted. The presence of such conditions was determined based on review of clinical diagnoses and laboratory reports. The length of follow-up and the presence of interval ischemic events were obtained.

Results

Out of a total of 180 patients diagnosed with MMD or MMS, 33 were found to have a concomitant pro-thrombotic condition (18.3%). Of 147 patients with MMD, 23 were found to have a pro-thrombotic condition (15.6%). There were 10 out of 33 total patients (30.3%) in the MMS cohort that had a concomitant pro-thrombotic condition. There were no differences in specific pro-thrombotic conditions between MMD and MMS cohorts. There were no differences in future ischemic events between moyamoya patients with pro-thrombotic tendencies compared to those without (13.3% versus 23.7%, respectively, P = 0.32), and this was also true for patients who underwent revascularization procedures (22.6% versus 29.4%, P = 0.76). There was also no difference in prevalence of revascularization graft occlusion between moyamoya patients with pro-thrombotic tendencies and those without at follow-up (1.2% versus 5.9%, P = 0.31).

Conclusions

It is likely that both MMD and MMS are associated with or predispose to pro-thrombotic conditions in adult patients, although the clinical and surgical significance is uncertain. Further study is needed in order to further elucidate and characterize this potential association.

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Abbreviations

MMD:

moyamoya disease

MMS:

moyamoya syndrome

TIA:

transient ischemic attack

DM:

diabetes mellitus

HLD:

hyperlipidemia

HTN:

hypertension

CAD:

coronary artery disease

MTHFR:

methylenetetrahydrofolate reductase

DVT:

deep vein thrombosis

Homozy:

homozygous

Heterzyg:

heterozygous

Ab:

antibody

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Correspondence to Anthony Larson.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the Mayo Clinic and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Approval from the Institutional Review Board was obtained prior to initiation of this study. For this type of study formal consent is not required.

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Larson, A., Rinaldo, L., Lanzino, G. et al. High prevalence of pro-thrombotic conditions in adult patients with moyamoya disease and moyamoya syndrome: a single center study. Acta Neurochir 162, 1853–1859 (2020). https://doi.org/10.1007/s00701-020-04420-8

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