From the Guidelines
Spontaneous cervical carotid artery dissection is primarily caused by underlying arteriopathy conditions, such as fibromuscular dysplasia, Ehlers-Danlos syndrome, and Marfan syndrome, which weaken arterial walls, as well as mild trauma from everyday activities and hypertension, as supported by the 2021 guideline for the prevention of stroke in patients with stroke and transient ischemic attack 1. The causes of spontaneous cervical carotid artery dissection can be multifactorial, including:
- Underlying connective tissue disorders, such as fibromuscular dysplasia, Ehlers-Danlos syndrome, and Marfan syndrome, which can weaken arterial walls, as noted in the 2011 ASA/ACCF/AHA/AANN/AANS/ACR/ASNR/CNS/SAIP/SCAI/SIR/SNIS/SVM/SVS guideline on the management of patients with extracranial carotid and vertebral artery disease 1.
- Mild trauma from everyday activities, such as coughing, sneezing, or turning the head suddenly, which can trigger dissection in predisposed individuals, as mentioned in the guidelines for the prevention of stroke in patients with stroke or transient ischemic attack 1.
- Hypertension, which places additional stress on arterial walls, as highlighted in the 2011 guidelines for the prevention of stroke in patients with stroke or transient ischemic attack 1. Other potential causes and risk factors include:
- Infections, particularly upper respiratory infections, which may cause inflammation that weakens arterial walls
- Genetic factors, with family history increasing risk
- Environmental factors, such as recent migraine headaches, oral contraceptive use, and smoking, which also contribute to risk
- Certain medications that affect blood vessel integrity, which might increase susceptibility
- Seasonal variations, with higher incidence in fall and winter, as observed in some studies The pathophysiology of spontaneous cervical carotid artery dissection involves blood entering the arterial wall through the tear, creating a false lumen that can restrict blood flow, form clots, or cause the artery to bulge, as explained in the 2011 ASA/ACCF/AHA/AANN/AANS/ACR/ASNR/CNS/SAIP/SCAI/SIR/SNIS/SVM/SVS guideline on the management of patients with extracranial carotid and vertebral artery disease 1. Understanding these causes is crucial for identifying at-risk patients and implementing preventive measures in those with predisposing conditions, as emphasized in the 2021 guideline for the prevention of stroke in patients with stroke and transient ischemic attack 1.
From the Research
Causes of Spontaneous Cervical Carotid Artery Dissection
The causes of spontaneous cervical carotid artery dissection are multifactorial and can be attributed to various factors, including:
- Environmental factors serving as possible triggers in patients who have a genetic predisposition to dissection formation 2
- Underlying arteriopathy related to an extracellular matrix defect, which could predispose to dissection 3
- Triggering factors such as minor trauma and infection 3
- Abnormalities suggesting an underlying arteriopathy, such as:
- Arterial redundancies
- Intracranial aneurysms
- Aortic root dilatation
- Common carotid artery distensibility increase
- Fibromuscular dysplasia
- Inherited connective tissue disorders
- Ultrastructural dermal connective tissue abnormalities 3
- Other factors associated with dissection, such as:
- Migraine
- Hyperhomocysteinemia
- Alpha-1 antitrypsin deficiency, suggesting arterial wall fragility secondary to hyperactivity of some proteases 3
- Risk factors, including:
- Neck trauma
- Recent infection
- Family history
- Smoking
- Hypertension
- Oral contraceptives
- Migraine headaches
- Connective tissue disease 4
- Genetic mutations, such as a mutation in exon 6 of the COL3A1 gene, confirming the diagnosis of vascular Ehlers-Danlos syndrome (vEDS) 5