An Overview of Carotidynia

Carotidynia (also called Fay Syndrome) is a medical term used to describe a rare idiopathic pain syndrome affecting the neck and face. It's referred to as an "idiopathic" syndrome because the symptoms arise spontaneously for unknown reasons. It is a somewhat controversial condition, as it is not clear if it is a disorder or a symptom of another condition.

The pain and tenderness described in carotidynia typically only affects one side (unilateral) of the neck, near the carotid artery. Carotidynia may be linked to migraines in some cases, but pain in the region can also be caused by more serious conditions.

Idiopathic carotidynia is characterized by neck tenderness and pain, typically where the carotid artery branches (bifurcates). The carotid arteries provide the head's blood supply and run along both sides of the neck. However, pain from carotidynia typically only occurs on one side.

The pain may be sudden and severe—people often describe it as a throbbing pain. It usually occurs in the absence of any trauma to the head or neck, and the pain is generally not associated with a recent infectious illness.

Patients may also have some swelling or fullness at the site of carotid bifurcation. Their carotid pulse may be more pronounced. These patients usually don't have fever or malaise and they often are employed in stressful professions.

Although the condition was first described in 1927, the medical community is still divided as to whether carotidynia is a disorder on its own or if it's a symptom of another condition. In some cases, the syndrome occurs in people who get migraine headaches. Neck pain and tenderness in the region can also occur in people who have carotid artery disease.

Severe pain can also be a sign of more serious conditions, such as giant cell arteritis.

The exact cause of carotidynia is unknown. Experts don't agree about whether the pain syndrome represents a distinct but uncommon condition, or if it's a symptom of other conditions commonly affecting the head, neck, and jaw. Some researchers have suggested it may be less confusing to use the term "caroditis," but this has not been widely adopted.

Inflammation appears to play a role in the development of the syndrome—biopsy specimens of patients with this condition have been shown to have cells known as lymphocytes, which indicate chronic inflammation. However, the exact mechanism for the inflammation is not well understood and research on the subject is limited.

When imaging studies are used to evaluate people with sudden, unexplained, unilateral neck pain and tenderness over the carotid artery, those who are ultimately diagnosed with carotidynia often have inflammation, swelling, or thickening of the artery. However, carotidynia is differentiated from other potential causes of neck pain because there is no structural abnormality present in the neck, jaw, or artery.

The diagnosis of carotidynia is usually made after all other potential causes for sudden, severe neck pain near the carotid artery have been ruled out. Medical professionals sometimes refer to this as a "diagnosis of exclusion."

If a person is experiencing neck pain, there are several tests that might be ordered to rule out various conditions that can cause the pain, some of which are serious and potentially life-threatening.

Tests that may be ordered include:

• a physical exam to feel (palpate) the neck.
• MRI, MR angiogram, CT scan, CT angiogram, ultrasound of the head and neck, and most recently PET-CT.
• blood tests to look for viruses, bacteria, or other abnormalities.

When the neck is felt during a physical exam, it may be very painful. Imaging of the neck tissue may be normal as well. In some cases, signs of inflammation near the carotid artery have been reported in patients later diagnosed with carotidynia. Also, the area may appear fuller or more swollen than the other side.

Blood tests, including those that look for markers of inflammation, are often normal.

While it can be very painful, people usually recover completely from carotidynia without any longterm problems. Pain usually begins to improve within a week.

There is no specific treatment for carotidynia, but medications to treat pain and inflammation (such as aspirin and ibuprofen) can be helpful. Most patients can take these medications in over-the-counter (OTC) doses by mouth. Some patients have found success in treating the pain with drugs called calcium channel blockers.

If pain is severe and does not respond to lower doses, a doctor may prescribe stronger doses or administer a shot to help.

In some cases, steroids such as prednisone have been proven effective at reducing inflammation associated with carotidynia. However, these medications should not be taken for a long time. If a person has been prescribed steroids, she will need to work closely with her doctor to reduce the dose and stop (taper) the drugs safely.

When carotidynia occurs in association with migraines, treatment and therapies typically used for migraine headaches have proven effective at reducing associated pain in some cases.

Carotidynia is characterized by neck tenderness and pain, typically where the carotid artery is. The pain can be sudden and severe. Diagnosis involves the exclusion of other conditions—including injury or illness. Carotidynia usually resolves without any long-term issues. In most cases, it can managed with medications for pain and inflammation. While carotidynia is generally not serious and does not tend to recur, any sudden, severe neck pain should be evaluated by a medical professional.