What is Carotidynia (Fay syndrome)?
Carotidynia, also known as Fay syndrome or TIPIC syndrome, is a very uncommon blood vessel disorder that causes one-sided neck and face pain. It falls under the category of ‘idiopathic neck pain syndromes’, meaning its origin is unknown. A key feature of this condition is pain at the spot where the carotid artery, a major blood vessel in the neck, splits. The discomfort worsens when you move your head, eat, yawn, cough, or swallow. The artery attached to the pain area may also beat faster. Carotidynia mostly affects one side, but there have been instances where it afflicts both sides.
Patients typically describe these episodes as neck pain that suddenly comes and goes, with each instance lasting about a week or two. It’s common for these episodes to reoccur every one to six months.
What Causes Carotidynia (Fay syndrome)?
The exact cause of carotidynia, a condition that causes neck pain and tenderness, is unknown. The condition was first described in 1927, as an unusual radiating pain in the neck that was felt when touching the spot where the carotid artery splits in two.
In 1988, the International Headache Society (IHS) decided to categorize carotidynia as a kind of headache if it showed certain features: Pain, swelling or an increased pulse when pressure is applied; no structural causes for the carotid pain; nerve pain in the head and neck that goes away within 14 days; and pain on one side of the neck that may spread to the head.
In 1994, experts argued that carotidynia wasn’t a disease but a painful symptom that could be attributed to migraines, viral throat infections, or carotid artery disease. In the same year, some argued that carotidynia couldn’t be properly diagnosed since none of the criteria proposed by the IHS in 1988 were consistently seen in reported cases. Based on scientific evidence from 2017, it was suggested that a syndrome called “thickened wall and infiltration of the perivascular fat involving the carotid bifurcation” or TIPIC – should be included under headaches in the International Classification of Headache Disorders (ICHD). Interestingly, it was also proposed that TIPIC syndrome should be used instead of carotidynia based on current research.
Although carotidynia was once classified as a type of migraine, it was removed from the list of headaches by the IHS in 2004 amidst controversy. It is now thought to be not a migraine but part of a vague neck pain syndrome. Modern imaging techniques have shown it to be an inflammation of the external layer of the blood vessels, and thus, the term transient perivascular inflammation of the carotid artery (TIPIC) syndrome has been recently adopted. This syndrome doesn’t classify as inflammation of the blood vessels, because carotidynia only involves the external layer of the blood vessel, whereas vasculitis involves inflammation of all layers of the vessel wall. Also, vasculitis affects vessels throughout the body, whereas carotidynia only involves the carotid artery.
In rare instances, carotidynia can be associated with different types of headaches outside the skull, cancer of the nasopharynx, anti-cancer treatments, and traveling to high altitudes.
Risk Factors and Frequency for Carotidynia (Fay syndrome)
Carotidynia, a rare condition, is often not well-documented, making its exact frequency hard to establish. However, a study revealed its occurrence to be about 2.8% in patients who come in with sudden neck pain. Generally, it has been observed in people mainly in their 40s and 50s and is reportedly more common in women than in men with a 1.5 to 1 ratio.
- Carotidynia is a rare condition, and its exact prevalence is hard to pinpoint.
- It seems to affect about 2.8% of patients who have severe neck pain.
- Most people who get this condition are in their 40s and 50s.
- Women are more likely to get it than men, with the ratio being 1.5 to 1.
Signs and Symptoms of Carotidynia (Fay syndrome)
Generally, a diagnosis can be made through a detailed health history and a physical exam. Patients often describe experiencing episodes of neck pain, which may sometimes radiate to the head. Surprisingly, these patients do not report signs usually associated with an infection, like chills, lymph node swelling in the neck, skin redness, or ear or throat pain. In some cases, the neck condition shows up after a person has recovered from an upper respiratory tract infection, which could imply that an immune reaction might be involved.
In the physical exam, doctors thoroughly check the ears, nose, and throat, and also look for any swollen lymph nodes in the head and neck area. They also feel the thyroid to see if it’s tender and check for any masses in the neck that may or may not pulse with the heartbeat. One unique feature of this condition is that individuals often have point tenderness on the side of the neck just above where the carotid artery splits. Touching the carotid artery may intensify this pain, causing it to spread to the ear on the same side. In some patients, a sound, like a whoosh, may be heard in time with the heartbeat when examining the carotid artery.
While most patients show no signs of neurological issues, a minority do report neurological symptoms. As such, the potential for this condition to affect the nervous system can’t be entirely ruled out. Other patients might also report feelings of anxiety. This could be due to the delay in getting a definitive diagnosis and worry about some potentially serious conditions, like a tear in the carotid artery or tumors in the head and neck.
Testing for Carotidynia (Fay syndrome)
Carotidynia, a type of neck pain, is typically diagnosed after other causes have been ruled out. It’s important to note that blood tests such as a complete blood count (CBC), thyroid function tests, erythrocyte sedimentation rate (ESR) which measures inflammation, and C-reactive protein (CRP) which also checks for inflammation in the body, will usually come back normal.
Images of the neck are required to eliminate other possible causes of neck pain. The initial test recommended is a neck ultrasound, which can reveal any abnormalities in the carotid wall, the area where you might experience discomfort. The ultrasound will typically show a thickening of the wall, slight narrowing and outward extension of the carotid wall, but it won’t disrupt the blood flow. This is a unique feature which helps distinguish carotidynia from other conditions, such as carotid artery atherosclerosis that includes plaque accumulation and calcification.
Other imaging techniques, such as computed tomography (CT) scans, digital subtraction arteriography, positron emission tomography (PET) scans, and magnetic resonance imaging (MRI), are not usually recommended. This is because they offer little extra benefit and can expose patients to radiation, or the use of IV contrast agents. These tests are typically only ordered if an ultrasound isn’t conclusive, or if the diagnosis isn’t clear. However, if a younger patient presents with certain neurological deficits consistent with a carotid artery dissection, a multidetector CT angiography may be beneficial. An MRI could be ordered for older patients if there’s suspicion of a condition known as giant cell arteritis, especially if they have raised levels of ESR or CRP.
Eventually, a PET scan may be beneficial if the diagnosis remains uncertain, even after an ultrasound, CT, and MRI. With a PET scan, it can be identified if the region of the carotid bifurcation, where the carotid artery splits into internal and external arteries, presents an increase in glucose metabolism. This alongside other factors like the response to steroid treatment can aid in the diagnosis of carotidynia.
A key characteristic of carotidynia is that these changes, seen through imaging, get better over time. Subsequent ultrasounds over the next few days demonstrate a reduction in the abnormalities to the carotid wall. In addition, PET scans can show a reduction in glucose activity within six months. This is consistent with the proposed renaming of the condition to transient perivascular inflammation of the carotid artery (TIPIC) syndrome.
There are other potential tests that can be used, like checking levels of serum amyloid A and soluble intracellular adhesion molecule-1 (ICAM-1), but these aren’t typically not used because their effectiveness is still under study. It’s crucial to also rule out other potential diagnoses that could cause neck pain such as infection, peritonsillar abscess, or giant cell temporal arteritis.
Treatment Options for Carotidynia (Fay syndrome)
Carotidynia, also known as a pain in the neck area due to inflammation of the carotid artery, typically clears up on its own within a week or two. Therefore, treatment often involves managing the pain rather than trying to cure the condition. Rest, reassurance, and taking painkillers like nonsteroidal anti-inflammatory drugs (NSAIDs) or aspirin are usually recommended initially.
If the pain continues, doctors may prescribe a short course of corticosteroids, which are medicines used to reduce inflammation. Other medications, including calcium channel blockers, selective serotonin reuptake inhibitors such as fluoxetine, and benzodiazepines, have also been reported to help manage the symptoms.
In the past, medications commonly used to treat migraines, such as propranolol, tricyclic antidepressants, ergotamine, and methysergide, were often used in treating carotidynia. However, this became less common after carotidynia was removed from the International Classification of Headache disorders in 2004.
Still, a more recent case report suggested that one such kind of anti-migraine medication, almotriptan, could effectively relieve the symptoms of carotidynia. Beyond medication, managing stress can also prove beneficial in dealing with this condition.
What else can Carotidynia (Fay syndrome) be?
If you are experiencing pain in the side of your neck, there are a number of possible medical issues that could be causing it. Two of the main ones, which involve the blood vessels, are carotid dissection and giant cell arteritis. In a carotid dissection, the pain is often accompanied by certain neurological symptoms like drooping eyelids, constricted pupils, and an inability to sweat on one side of the face. However, typically, there are no neurological symptoms with carotidynia. There are also many other potential causes of this kind of pain:
- Infections: This could include infections in the area around the throat, mouth, and glands such as lymphadenitis, sialadenitis, peritonsillar abscess, and thyroiditis
- Neurological issues: Conditions such as migraines or trigeminal neuralgia could be the cause
- Cancer: If there’s a cancerous growth in the head or neck, that could lead to the pain
- Musculoskeletal problems: Issues such as cervical spondylitis, temporomandibular joint syndrome, and Eagle syndrome could result in neck pain
- Vascular issues: Apart from carotid dissection, conditions such as carotid body tumor, carotid aneurysm, fibromuscular dysplasia, acute carotid occlusion, and large-vessel vasculitis could cause pain in the carotid artery
What to expect with Carotidynia (Fay syndrome)
Each instance of carotidynia, which is pain in the neck due to an inflamed artery, usually goes away by itself in about 7 to 14 days. Usage of NSAIDs (Non-Steroidal Anti-Inflammatory Drugs) and corticosteroids, a type of medicine, can help the symptoms go away faster. However, patients can expect the pain to come back every 1 to 6 months.
Possible Complications When Diagnosed with Carotidynia (Fay syndrome)
Carotidynia, a condition traditionally seen as resolving naturally in a week or two, has recently been linked to blockages of the carotid artery and brain-related complications in newer studies. The hard-to-define nature of this medical issue might lead patients to undergo needless and intense tests – both invasive and non-invasive. This could expose them to high levels of radiation from CT scans and the powerful magnetic fields of MRI scans.
Misdiagnoses may also occur, with patients wrongly marked as having a disease called “giant cell temporarily arteritis.” This could lead to them experiencing possibly harmful biopsies of the temporal artery and other surgery-related complications. The long, often expensive, series of tests might also lead to anxiety because of not finding a clear diagnosis. So, to avoid the risks of unnecessary tests, carotidynia should be recognized as a medical condition in its own right.
Potential Risks:
- Exposure to high radiation of CT scans
- Exposure to strong magnetic fields of MRI scans
- Unnecessary invasive procedures
- Possible complications from a temporal artery biopsy
- Anxiety from prolonged and expensive testing
- Lack of a definitive diagnosis
Preventing Carotidynia (Fay syndrome)
Some patients suffering from a condition called Carotidynia, which affects the carotid artery in the neck, often experience anxiety. It’s important to reassure them that this condition is generally not as serious as other similar conditions like a tear in the carotid artery, temporal arteritis (an inflammation of blood vessels in and around the brain), or neck tumors. Nevertheless, patients should be aware that their symptoms may come back. In fact, most patients experience a return of symptoms within 1 to 6 months. Healthcare providers should monitor these patients, and make sure they understand that if Carotidynia does return, it is usually not harmful.
