Abstract
Dolichoectasia is an arterial disease that causes dilatation and/or tortuosity of the affected vessel. The prevalence of dolichoectasia increases with age, and this disease is also associated with other traditional cardiovascular risk factors. Multiple pathophysiological processes might lead to the development of dolichoectatic vessels, and activation of metalloproteinases and irregular turbulent blood flow seem to cause irreversible disruption of the internal elastic lamina. Intracranial dolichoectasia commonly presents with ischemic or hemorrhagic stroke, and/or cranial neuropathies. The posterior circulation is more frequently affected by the dolichoectatic process than the anterior circulation. A positive diagnosis of dolichoectasia requires visual assessment of vessel shape and, if the posterior circulation is affected, application of Smoker's criteria. Reproducible criteria that aid diagnosis of dolichoectasia in the anterior circulation are lacking. No specific treatment for dolichoectasia exists, and the surgical and medical therapies that have been used to treat this condition have not been systematically evaluated. More evidence is needed to better understand the underlying dilatatory artheriopathy that causes this disease, and to determine whether patients with dolichoectasia might benefit from early diagnosis. In this article, we provide a comprehensive review of current knowledge regarding dolichoectasia, and highlight gaps in our knowledge to aid future research.
Key Points
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Dolichoectasia is a disease that can affect both intracranial and extracranial arteries, and can be asymptomatic or manifest with compressive and/or vascular symptoms
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Dolichoectasia is associated with traditional cardiovascular risk factors such as hypertension, old age and male sex, but can also be associated with nonatherosclerotic diseases
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Disruption of the internal elastic lamina and subsequent weakness and deformation of the arterial wall are characteristic features of dolichoectasia
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Limited evidence exists to recommend a specific therapy for dolichoectasia, although treatment of the underlying risk factors seems to be the safest approach
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Future studies are needed to address multiple questions regarding the physiopathology, treatment and prognosis of dolichoectasia
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J. Gutierrez researched the data for the article, provided substantial contributions to discussions of the content, and contributed to writing the article and to review and editing of the manuscript before submission. C. B. Wright provided substantial contributions to discussions of the content, and contributed to writing the article and the review and editing of the manuscript before submission. R. L. Sacco provided substantial contributions to discussions of the content and to review and editing of the manuscript before submission.
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Patient characteristics of selected studies of dolichoectasia (DOC 105 kb)
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Gutierrez, J., Sacco, R. & Wright, C. Dolichoectasia—an evolving arterial disease. Nat Rev Neurol 7, 41–50 (2011). https://doi.org/10.1038/nrneurol.2010.181
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DOI: https://doi.org/10.1038/nrneurol.2010.181
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