Carotid Artery Dissection


Article Author:
Steven Goodfriend
Prasanna Tadi


Article Editor:
Ron Koury


Editors In Chief:
William Gossman


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes


Updated:
5/16/2019 8:50:03 PM

Introduction

Carotid artery dissection is a condition whereby the layers of the carotid artery are spontaneously separated. This potentially compromises blood flow to certain areas of the brain and can lead to a stroke. Furthermore, it can occur extracranially or intracranially and can lead to subarachnoid hemorrhage or brain ischemia. It is the most common cause of strokes in younger patients, and the importance of making a timely diagnosis is paramount to minimize potential morbidity and mortality of the disease. [1] There is a huge variation in the presenting signs and symptoms of this disease which makes it extremely difficult to diagnose on initial presentation.[2]

Etiology

Carotid artery dissection occurs spontaneously when a tear occurs in the intimal layer of the carotid artery creating an intramural hematoma. This tear can be spontaneous or caused by trauma. [3] The intramural hematoma causes the stenosis and eventual thrombus formation. Traumatic dissection can either be by blunt or penetrating trauma. Blunt trauma can be significant (e.g., motor vehicle collision), or it can seem minimal (e.g., chiropractic manipulation being the most classic example)[4]. A motor vehicle accident where there is rapid deceleration with simultaneous neck hyperextension and rotation may lead to an intimal tear of the carotid artery.[5] "Idiopathic" is the most common cause of spontaneous carotid dissections in which a family history of dissection significantly increases one's risk. [6][7][8] Marfan syndrome, Ehlers-Danlos Syndrome, fibromuscular dysplasia and other connective tissue disorders also increase the risk of this disease. An elongated styloid process, which is called Eagle Syndrome, can also cause a spontaneous internal carotid artery dissection.[9]

Epidemiology

Carotid artery dissections occur in all age groups. They accounts for 2.5% of all strokes. [10] It is a common cause of stroke in patients younger than 40 years old. In all young patients, 20% of cerebrovascular accidents (CVAs) are caused by a carotid artery dissection. The median age is in the mid-40s, and there seems to be a slightly higher incidence in males versus females.[2]

Pathophysiology

A sudden tear in the intimal layer of the carotid artery occurs due to the factors listed above. These may be due to trauma or they can be spontaneous. This tear allows blood to flow into this intimal layer of the vessel and allows a hematoma to form within the blood vessel wall. This is commonly known as a false lumen. As blood enters this false lumen, it causes stenosis which may lead to complete occlusion of the carotid artery. This is a dynamic process which may lead to stenosis of the vessel or dilatation of the artery. This depends on where the hematoma develops, either toward the intima or adventitia. This process may lead to a stroke from a complete vascular occlusion at the site of the dissection. It may also be a nidus for thrombus formation that may later migrate distally, causing a stroke or transient ischemic attack. If the vessel ruptures intracranially, it may lead to a subarachnoid hemorrhage. Also, a pseudoaneurysm may result from a carotid dissection.[11]

History and Physical

There is a tremendous amount of variation in the presentation of carotid dissection, hence making the diagnosis a challenge. [12] The presentation can range from an asymptomatic patient to one who presents with an acute stroke. Classic teaching describes a headache, facial or eye pain, and neck pain. The pain when present is usually on the ipsilateral side. A Horner's Syndrome may be present if a hematoma of the cervical artery compresses the adjacent sympathetic nerve fibers. When stroke-like symptoms are present with these symptoms, it can make the diagnosis somewhat less difficult. The family history of carotid dissection or connective tissue disorders may heighten one's suspicion. The presence of blunt or penetrating trauma is helpful to make the diagnosis.  Blunt trauma makes it more challenging to make the correct diagnosis as some can occur from minor trauma such as a chiropractic neck manipulation.  Unfortunately, many patients may not present with pain nor a mechanical trigger which further increases the challenge of making an accurate diagnosis.

Evaluation

As is common with many disease entities that are rare and can present with minor to more severe symptoms, one must have a higher index of suspicion to make this diagnosis. Unfortunately, having a too high index of suspicion in search of this disease may lead to overtesting in a vast array of patients that ultimately do not have a carotid artery dissection. A good history and physical is essential. Neurologic deficits should prompt one to consider this diagnosis especially if the patient is young and there is recent trauma. Getting a good medical and family history may aid in the diagnosis. Recent trauma to the neck with anterior neck pain is a clue. Neck pain and tenderness may be present. A bruit over the carotid artery may be heard in some patients with this disease. An expanding hematoma may be present if trauma has occurred.

If a cervical dissection is suspected, many diagnostic modalities may confirm the diagnosis. The and initial least invasive screening tool is a carotid ultrasound. Its sensitivity is not as good as a CT angiogram, and it does not allow for imaging of the intracranial vessels. A CT angiogram is more commonly ordered these days as it can be done concurrently with a brain CT that is looking for an acute stroke or intracranial bleed. A flame sign is classic that is tapering of the carotid artery can be seen. MRI and magnetic resonance angiography are reasonable alternatives if the patient has contraindications to a CT angiogram, but it is not as sensitive. Standard digital subtraction angiography was the most common means of making this diagnosis, but with the evolution of CT angiography, this imaging modality has become much less common from a diagnostic standpoint.

Treatment / Management

Treatment of a cervical dissection depends on many factors such as the cause (traumatic vs. spontaneous), and whether the patient has had a stroke.  Also, whether this dissection is intracranial or extracranial will alter the treatment plan. Active bleeding with hematoma expansion will also be a factor in deciding treatment.

If there are no contraindications, antiplatelets may be used, or more commonly, systemic anticoagulation may be used to minimize the risk of a stroke. Also, endovascular stenting may be performed on some of these patients, especially if there are contraindications to anticoagulation or if medical management fails. The one-year recurrence rate is 0-10%. 

Pearls and Other Issues

Carotid dissection can lead to minor symptoms or more commonly, to severe neurologic deficits and/or death. Prognosis is variable and depends on whether the diagnosis is made before the onset of stroke symptoms. All patients have a very high risk of stroke, intracranial bleeding, and are subjected to anticoagulation which has its set of risks.

Carotid dissection is a rare disease, and it is an extremely difficult diagnosis to make. The presentation can vary from minor symptoms to more severe life threatening symptoms, as discussed above. Treatment is aimed at minimizing the risk of stroke and worsening of symptoms.

Enhancing Healthcare Team Outcomes

Carotid artery dissection is a rare vascular disorder that has the potential to cause a severe stroke. Thus, it is best managed by a multidisciplinary team that includes a neurologist, emergency department physician, radiologist, vascular surgeon and an inermist. The diagnosis is made by CT scan but the management depends on patient symptoms and whether the disease in extra or intracranial. Asymptomatic patients may be treated with systemic anticoagulation but if there is a risk of bleeding or rupture, the patient may require surgery. Today a viable option is stenting. These patients should be monitored in a neuro ICU by nurses until they are hemodynamically stable and have no evidence of any neurological deficit. Patients need to be educated about blood pressure control as recurrence of dissection has been reported in up to 10% of cases. [13](Level V)

 


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Carotid Artery Dissection - Questions

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Which of the following is true regarding blunt trauma to the internal carotid artery?

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    Contributed by Scott Dulebohn, MD
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A patient with a traumatic cervical carotid artery dissection is unlikely to present with which of the following symptoms?



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A 40 year old weekend athlete is sent to his provider by his wife with unequal pupils and a droopy left eyelid. He was playing football on Sunday and received a harder hit than usual. MRI was abnormal. Which of the following is most likely?



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A 38-year-old male presents to the emergency department after a 3-day history of right-sided neck pain and headache. He has no significant past medical history. His headache started after a day at an amusement park where he rode several roller coasters. He had taken aspirin without relief but became concerned when he had a 10-minute episode of confusion associated with left-hand numbness and clumsiness. An exam shows mild, right ptosis, anisocoria with the right pupil being smaller, and left arm drift. What is the most likely diagnosis?



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A 38-year-old man presents to the emergency department after a 3-day history of right-sided neck pain and headache. He has no significant past medical history. He took aspirin without relief but became concerned when he had a 10-minute episode of confusion associated with left-hand numbness and clumsiness. The exam shows mild right ptosis, anisocoria with the right pupil being smaller, and left arm drift. Doppler ultrasonography reveals a thrombus and possible right-sided internal carotid artery dissection. What treatment will this patient most likely receive?



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Spontaneous extracranial internal carotid artery dissection most often causes injury to which of the following cranial nerves?



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What percentage of ischemic strokes in young patients are secondary to spontaneous dissection of the vertebral or carotid arteries?



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Which cranial nerve is most commonly affected by carotid dissection?



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A 25-year-old female with a history of classic migraines saw a chiropractor for manipulation. Three days later, the patient developed right-sided, anterolateral neck pain and headache. The patient then had right-eye blindness that lasted a few hours. An exam shows mild right tongue deviation, ptosis, and right miosis. What is the most likely diagnosis?



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A 39-year-old man presents with complaints of transient visual loss in the right eye. There is associated headache, facial, and neck pain on the same side. The ophthalmologist notices that there is a mild ptosis in the right eye, and pupil examination shows a smaller pupil in the same eye. Which of the following conditions has to be ruled out?



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A 29-year-old yoga enthusiast decided to paint a mural on her baby daughter's ceiling. The dust caused her to sneeze while stretching overhead to paint on a short ladder. Shortly after, she developed a migraine, transient poor vision, and a decreased taste sensation. What test will this young lady need in the emergency department?



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Carotid Artery Dissection - References

References

Cervical Artery Dissection: A Review of the Epidemiology, Pathophysiology, Treatment, and Outcome., Blum CA,Yaghi S,, Archives of neuroscience, 2015 Oct     [PubMed]
Carotid and vertebral artery dissection syndromes., Thanvi B,Munshi SK,Dawson SL,Robinson TG,, Postgraduate medical journal, 2005 Jun     [PubMed]
Delayed Stroke following Blunt Neck Trauma: A Case Illustration with Recommendations for Diagnosis and Treatment., Anyama B,Treitl D,Wessell J,Solomon R,Rosenthal AA,, Case reports in emergency medicine, 2017     [PubMed]
Carotid artery dissection: a rare complication of Eagle syndrome., Subedi R,Dean R,Baronos S,Dhamoon A,, BMJ case reports, 2017 Mar 13     [PubMed]
Caplan LR, Dissections of brain-supplying arteries. Nature clinical practice. Neurology. 2008 Jan;     [PubMed]
Hart RG,Easton JD, Dissections of cervical and cerebral arteries. Neurologic clinics. 1983 Feb;     [PubMed]
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Ojemann RG,Fisher CM,Rich JC, Spontaneous dissecting aneurysm of the internal carotid artery. Stroke. 1972 Jul-Aug;     [PubMed]
Chaves C,Estol C,Esnaola MM,Gorson K,O'Donoghue M,De Witt LD,Caplan LR, Spontaneous intracranial internal carotid artery dissection: report of 10 patients. Archives of neurology. 2002 Jun;     [PubMed]
O'Connell BK,Towfighi J,Brennan RW,Tyler W,Mathews M,Weidner WA,Saul RF, Dissecting aneurysms of head and neck. Neurology. 1985 Jul;     [PubMed]
Sherman DG,Hart RG,Easton JD, Abrupt change in head position and cerebral infarction. Stroke. 1981 Jan-Feb;     [PubMed]
Giroud M,Gras P,Dumas R,Becker F, Spontaneous vertebral artery dissection initially revealed by a pain in one upper arm. Stroke. 1993 Mar;     [PubMed]
Befera N,Griffin AS,Hauck EF, Endovascular repair of an acute symptomatic carotid artery dissection through the false dissecting carotid lumen. Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences. 2019 Feb;     [PubMed]

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