Dissection Syndromes
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Dissection occurs when blood extrudes into the connective tissue framework of a vessel wall, causing separation of the natural vessel layers. When a dissection occurs between intima and media layers, luminal narrowing or occlusion can occur. Dissection between the media and adventitia results in aneurysmal dilation, which can lead to rupture. Rupture of an artery with subsequent encapsulation of the extravascular hematoma results in pseudoaneurysm, which can lead to luminal narrowing. Dissection of the cervical and intracranial vessels is an uncommon but increasingly recognized condition.
The cervical (extracranial) internal carotid artery is affected in 75% of patients (usually approximately 2 cm distal to the bifurcation) and the extracranial vertebral artery in 15% of patients. The remaining cases usually involve the intracranial internal carotid artery, intracranial vertebral artery, middle cerebral artery, or basilar artery. Cervicocephalic dissections may occur spontaneously or secondary to major or minor trauma. In some patients, they are associated with an underlying arteriopathy. Fifteen percent of cases are bilateral, and one half of these occur in patients with underlying fibromuscular dysplasia.
The hallmark of dissection is hemorrhage within the vessel wall. In some patients, an intimal tear allows intravascular blood to communicate directly with the vessel wall cavity. In others, an intramural hematoma develops without a direct connection with the vessel lumen.
In extracranial carotid and vertebral dissections, hemorrhage into the medial-adventitial layers occurs most commonly. This occasionally causes the external vessel wall to bulge outward, forming a dissecting aneurysm that can compress local structures. In intracranial carotid and vertebral dissections, subintimal tears occur more commonly, leading to formation of intramural hematomas that protrude inward and narrow the vessel lumen. Most ischemic symptoms (85-95%) are caused by emboli from the site of the dissection, while the remainder are due to vessel narrowing with hemodynamic insufficiency (5-15%) or a combination of both.
Hospital-based series suggest that cervicocephalic dissections are responsible for 1–2.5% of ischemic strokes in the general population and for 5–20% of strokes in individuals younger than 45 years. In one community-based study, the average annual incidence of spontaneous cervical internal carotid artery dissections was 2.6 cases per 100,000. While improved imaging techniques and growing awareness of the disorder have led to increased recognition of these syndromes, mild cases likely will remain undiagnosed. Multiple dissections occur in 10%, with the most common being bilateral vertebrobasilar lesions. [1]
International frequency of dissection syndromes is similar to that in the United States.
Morbidity and mortality of cervicocephalic dissections vary according to the vessel and location of the dissection. Death rates for extracranial carotid and vertebral dissections are approximately 5-10%. In contrast, mortality rates for intracranial carotid and basilar dissections approach 70% or higher.
Persons of all ages may be affected; however, dissections occur more frequently in younger individuals. In extracranial carotid dissection, 70% of cases occur in persons aged 35–50 years. Intracranial carotid dissection tends to occur particularly in adolescents and adults younger than 30 years.
While some studies have reported that males and females are affected equally in extracranial carotid dissections, the Cervical Artery Dissection and Ischemic Stroke Patients (CADISP) group reported that cervical artery dissection was more common in men and men were older at onset. Intracranial dissections are more common in younger males than in females. Extracranial vertebral artery dissections and multiple vessel dissections are more common in women than in men.
No racial preponderance is demonstrated.
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Engelter ST, Dallongeville J, Kloss M, Metso TM, Leys D, Brandt T, et al. Thrombolysis in cervical artery dissection–data from the Cervical Artery Dissection and Ischaemic Stroke Patients (CADISP) database. Eur J Neurol. 2012 Sep. 19(9):1199-206. [Medline].
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Gaurav Gupta, MD Assistant Professor, Section Head, Endovascular and Cerebrovascular Neurosurgery, Fellowship Director, Endovascular Neurosurgery Fellowship (Site), Department of Surgery, Division of Neurosurgery, Rutgers Robert Wood Johnson Medical School
Gaurav Gupta, MD is a member of the following medical societies: American Academy of Neurology, American Association for the Advancement of Science, American Association of Neurological Surgeons, American College of Surgeons, American Heart Association, American Medical Association, Congress of Neurological Surgeons, Facial Pain Association, Society for Neuroscience, Society of NeuroInterventional Surgery
Disclosure: Nothing to disclose.
Arthur Carminucci, MD Resident Physician, Department of Neurological Surgery, Rutgers New Jersey Medical School
Disclosure: Nothing to disclose.
Sudipta Roychowdhury, MD Clinical Associate Professor of Radiology, Department of Radiology, Rutgers Robert Wood Johnson Medical School; Attending Radiologist/Neuroradiologist, University Radiology Group, PC
Sudipta Roychowdhury, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Neuroradiology, Medical Society of New Jersey, Radiological Society of New Jersey, Radiological Society of North America, Society of NeuroInterventional Surgery
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
Howard S Kirshner, MD Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Neurological Association, American Society of Neurorehabilitation, American Academy of Neurology, American Heart Association, American Medical Association, National Stroke Association, Phi Beta Kappa, Tennessee Medical Association
Disclosure: Nothing to disclose.
Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association
Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2; Executive Committee for the NINDS-funded DEFUSE3 Trial; Physician Advisory Board for Coherex Medical.
Chelsea S Kidwell, MD Professor, Department of Neurology, University of Arizona College of Medicine
Chelsea S Kidwell, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Society of Neuroimaging, National Stroke Association
Disclosure: Nothing to disclose.
William J Nowack, MD Associate Professor, Epilepsy Center, Department of Neurology, University of Kansas Medical Center
William J Nowack, MD is a member of the following medical societies: American Academy of Neurology, Biomedical Engineering Society, American Clinical Neurophysiology Society, American Epilepsy Society, EEG and Clinical Neuroscience Society, American Medical Informatics Association
Disclosure: Nothing to disclose.
Richard E Burgess, MD, PhD Assistant Professor, Department of Neurology, Georgetown University Hospital; Medical Director, Clinical Stroke Service
Richard E Burgess, MD, PhD is a member of the following medical societies: American Academy of Neurology and American Heart Association
Disclosure: Nothing to disclose.
Dissection Syndromes
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