- Carotidynia
is a symptom of unilateral vascular neck pain
which was first described by Temple Fay in 1927.
The cardinal physical finding is tenderness on
palpation of the carotid artery. The incidence
and prevalence of carotidynia are unknown. Most
authors agree that it is common but frequently
unrecognized. It may be two or three times as
common as cluster headaches
-
- Lovshin reported a series of 100 cases in
patients who ranged in age from 10 to 78 years
with a median age of 55 years. Female patients
were affected four times as frequently as male
patients. In Lovshin's series, as in many
others, no serious organic conditions were found
among patients presenting with carotidynia, and
all were treated symptomatically.
-
- We present three case reports, followed by
the diagnosis, possible causes, and various
treatment modalities for this syndrome.
-
- Case Reports
-
- Case 1. A 42-year-old man
presented with a history ofpain in the left side
of his neck for the previous 2 weeks. He was in
normal health except for a history of migraine
headaches, which had occurred once or twice a
year since he had been 18 years old. The patient
indicated that his pain was moderate in
intensity, transmitted slightly to the left ear,
and was exacerbated by swallowing. His most
recent headache had occurred approximately 1
month before the onset of the neck pain. No
abnormalities were found on examination of the
ears, nose, mouth, and throat. Indirect
laryngoscopy was normal. Although palpation of
the neck revealed no abnormal lymph nodes, organ
enlargement, masses, or deformities, the left
carotid artery was moderately tender just below
the bifurcation. The patient was treated for 2
weeks with a nonsteroidal anti-inflammatory
agent. The pain gradually diminished and
resolved, although he had mild recurrences for
several months.
-
- Case 2. A 30-year-old female
patient presented to the clinic with a 2-week
history of persistent pain and fullness in her
face. She had a history of mild episodes of
sinusitis and allergic rhinitis. Her vital signs
were: pulse 64 beats per minute, blood pressure
130/90 mg Hg, temperature 97.4°F, and
weight 121 lb. Physical examination showed
normal tympanic membranes. Her nasal mucosa was
edematous and red, and the area over both
maxillary sinuses was tender to percussion.
Neck, chest, cardiovascular, and abdominal
examinations were all normal. She was treated
with amoxicillin 500 mg three rimes a day for 14
days and a combination antihistamine and
decongestant.
-
- She returned to the clinic 1 month later
complaining of persistent pain in the left side
of her neck and in her left ear. The sinusitis
had subjectively resolved by this time. Vital
signs were: pulse 88 beats per minute, blood
pressure 120/70 mm Hg, and temperature
96.0°F. On examination, the tympanic
membranes were found to be clear. There was no
sinus tenderness to percussion. Nose, throat,
and oral examinations were normal. Her neck was
supple with no adenopathy, organomegaly, or
masses. A tender, throbbing carotid artery on
the left side was noted. The remainder of the
examination was normal. She was treated with a
nonsteroidal anti-inflammatory agent, and the
pain resolved during the next 1 to 2 weeks.
-
- Case 3. A 36-year-old woman
presented to the emergency department with a
3-day history of sore throat, fever, stiff neck,
and right-sided headache extending from behind
the angle of the jaw to the entire right
hemicrania.
-
- Since the age of 18 years, the patient had
experienced episodes of severe, usually
unilateral pounding headaches, which were
accompanied by vomiting and sometimes preceded
by scintillating contralateral visual scotomata.
The headaches frequently resolved with
sleep.
-
- The patient's temperature was 104°F.
She appeared to be in pain and was photophobic.
The throat was deeply hyperemic and the tongue
thickly coated. The right carotid bulb pulsed
prominently and was exquisitely tender to touch.
The sternomastoids and cervical trapezius were
moderately tense and tender, and flexion of the
neck was limited to 45°. There was no
adenopathy or thyromegaly. The neurological
examination was unremarkable.
-
- The white blood count was 8200/p.l, with 30
segmented neutrophils, 47 polymorphous nuclear
leukocytes, 19 lymphocytes, and 4 monocytes.
Spinal fluid protein was 31 mg/dl, glucose 77
mg/dl, and cell count 7 mononuclears/l. The
sedimentation rate was 63. A repeat
sedimentation rate at 2 weeks follow-up was 13.
All cultures and other laboratory tests were
negative.
-
- The patient was treated with intravenous
antibiotics and narcotic analgesics. The sore
throat and fever gradually resolved during the
ensuing 5 days. The headache and neck pain
resolved with analgesia within the first 48
hours, but an exacerbation occurred on the day
before discharge, which again resolved with
symptomatic treatment. She continues to suffer
from frequent episodes of pain that involve
either or both sides of the head or neck and
often require narcotic analgesics.
-
- Discussion
-
- Clinical Characteristics
-
- The pain of carotidynia is most often
unilateral and localized to the neck, although
radiation to the face, ear, or malar region is
not uncommon. It is frequently described as dull
and throbbing in character and continuously
present, although day-to-day or hour-to-hour
exacerbations and remissions are common.
Severity varies from mild to agonizing, and the
pain is frequently aggravated by swallowing,
yawning,
coughing, sneezing, or elevating the head while
moving it toward the contralateral side. A
history of migraine may be present (eg, the
first and third cases). In other cases, a
history of previous pharyngitis, tonsiitis,
upper respiratory tract infection, oral
conditions, or recent dental procedures is
reported (cg, the second and third cases).
Anxiety or fear of cancer is often
present.3'4
-
- Physical examination is usually normal
except for mild to severe tenderness and
sometimes prominent pulsing at the carotid
artery bifurcation. Tenderness has also been
reported over the proximal 6 cm of the internal
carotid or the facial artery. Fay's sign was
originally described as follows: "If the thumbs
are placed on the common carotid artery just
below the bifurcation, and the structures
pressed back against the transverse cervical
processes with a rolling movement, a severe
reaction ofpain is produced on the side of the
atypical neuralgia. This response I have termed
"carotidynia."
-
- Differential Diagnosis
-
- Many other conditions, some potentially
serious, can cause unilateral neck pain. Most of
these conditions may be excluded by a careful
examination of the head and neck, supplemented
when indicated by laboratory or radiology tests.
Acute pharyngitis, peritonsillar abscess, acute
sinusitis, dental abscess, temporomandibular
joint syndrome, and cancer of the tongue,
salivary glands, or larynx can be excluded by a
combination of observation, palpation, and
percussion of the structures of the mouth, face,
and throat accompanied by direct or indirect
laryngoscopy.
-
- The pain in Eagle's
syndrome (facial pain caused by an elongated
styloid process) can be reproduced by finger
pressure along the base of the tongue. The
tenderness of thyroiditis and tracheitis are
located more centrally and symmetrically in the
neck. It is important to exclude anterior
cervical adenopathy by palpation, as this is
probably the most common incorrect diagnosis.
Cervical muscle spasm can be excluded by
palpation of the muscles involved. The
distinctive patterns of radiation of cervical
disc disease and degenerative cervical arthritis
coupled with the absence of Fay's sign should
direct attention to these conditions.
-
- Pathogenesis and Origin
-
- Carotidynia is a symptom based on a
characteristic complaint accompanied by a unique
physical finding. The International Headache
Society classifications include carotidynia
(idiopathic). However, in the literature,
carotidynia is not described as a clinical
entity in and of itself, but rather as a symptom
of another process. We concur with the authors
of The Headaches" that "ideopathic carotidynia
has not been described and that it is a syndrome
of neck pain." Since it is not a disease, there
are several possible causes.
-
- A local process involving the carotid artery
appears to cause local pain and tenderness as
well as referred pain to the mandible, face,
eyes, ear, and head. The pain is produced by
stimulation of the nerve endings of the carotid
plexus as described by Fay. He reproduced the
pain's characteristic radiation patterns by
electrical stimulation (Figure), and based on
his observations ofpatients undergoing nerve
resection for intractable pain, deduced that the
vagus nerve and the cervicothoracic spinal nerve
roots must be the neurological pathways to the
brain.
-
- Reported causes of carotidynia include
migraine, viral or postinfection, giant cell
(temporal) arteritis, carotid artery dissection,
carotid artery aneurysm and total carotid artery
occlusion. The first two causes are the most
common and the most benign. Treatment and
prognosis depend on the cause. Physicians can
distinguish benign causes from those that are
serious by inquiring about neurological
symptoms, performing a neurological examination,
and considering the results of a sedimentation
rate. Unusual or suspect presentations may
require further laboratory or imaging
studies.
-
- MIGRAINE
-
- The most commonly ascribed cause is
migraine. Lovshin noted a high incidence of
vascular headache in both family and personal
histories before the onset of carotidynia. He
also observed that many of his patients had a
"migraine personality" (sensitive,
conscientious, and compulsive). His patients
tended to suffer most from carotidynia when
fatigued, frustrated, or under stress, but also
during periods of relaxation after stress.
Raskin and Prusiner9 made the important
observation that Fay's sign is positive in more
than 50% of patients experiencing at least one
vascular headache per week. The association is
so striking as to lead one author to advocate
that "carotidynia is such a frequent
accompaniment of migraine that part of the
routine examination of these patients should
include palpation of the neck."
-
- Carotidynia associated with migraine tends
to recur over periods of months to years with
exacerbations sometimes lasting up to several
weeks. The pain in these cases is generally
described as dull and deep-seated, aggravated by
swallowing, stooping, or straining, and
accompanied by a mild earache. The pain in this
variant of carotidynia usually responds to drugs
commonly prescribed for migraine, such as
ergonovine, tricyclic antidepressants, beta
blockers, calcium channel blockers, or
methysergide.
-
-
- INFECTION OR POSTINFECTION
-
- Carotidynia is also known to follow cases of
pharyngitis, infected aphthous ulcers, and viral
upper respiratory tract infection.
Roseman'° reported a series of 33 younger
patients (mean age, 37 years) with first attacks
of carotidynia. The patients were equally
divided between male and female patients. These
patients frequently presented with the complaint
of "sore throat." The symptoms were otherwise
much like those previously described, except
that they were usually severe or moderately
severe and the patients frequently presented
with considerable anxiety. There was no family
or personal history of migraine, and the
symptoms were self-limited, lasting an average
of 11.6 days. The recurrence rate was 10%. One
third of these patients had objective evidence
of pharyngeal inflammation and another third had
symptoms suggesting upper respiratory tract or
other viral infection. Fever was usually absent
and laboratory tests were usually normal. The
causative agent in these cases was unknown but
suspected to be viral. Neither corticosteroids
nor antibiotics modified the course, and the
treatment was supportive.
-
- GIANT CELL (TEMPORAL) ARTERITIS
-
- Carotid arteritis is a less likely but more
serious diagnostic possibility. Although cranial
arteritis occurs almost exclusively in patients
over 55 years old, carotid arteritis frequently
occurs in younger patients. Swann'6 reported a
series of 17 patients with giant cell carotid
arteritis, three of whom were in their early 20s
and only one of whom was over the age of 50
years. All had tender carotid arteries, many had
low-grade fever and malaise, and all responded
promptly to corticosteroids. Two other case
reports described patients with this variant of
giant cell artentis who were younger than 50
years of age.
-
- Most cases of giant cell (temporal)
arteritis resolve spontaneously within 2 to 30
months. Neurological damage, including visual
loss, is usually permanent, and if untreated,
has an overall mortality rate of 12%. Therefore,
an erythrocyte sedimentation rate is mandatory
for all patients presenting with carotidynia
regardless of age. Corticosteroid therapy is the
treatment of choice. The diagnosis can sometimes
be confirmed by biopsy, but temporal artery
biopsy is usually negative. Paulley and Hughes
favor facial artery biopsy if needed to confirm
the diagnosis.
-
- CAROTID ARTERY ANEURYSM
-
- Chambers and colleagues reported two
patients with carotid aneurysms who presented
with carotidynia. Their review of the literature
up to that time (1981) noted that in 39 of 113
previously reported cases of carotid aneurysm,
the patients had suffered from head and neck
pain. They cited Countee's observation that
carotidynia was "the most common presentation"
of carotid aneurysm.
-
- A carotid artery aneurysm can be located
anywhere along the common or internal carotid
arteries and may manifest solely as a tender
carotid artery. A mass may or may not be
palpable, and even if present, it may not be
pulsatile. Arteriography is the most reliable
means of reaching a definitive diagnosis.
Although arteriosclerosis and trauma are the
most common causes, Marfan's syndrome, cystic
medial necrosis, ionizing radiation, pyogenie
infections, previous carotid artery surgery,
congenital defects, and syphilis are also
associated. Although rupture of the artery is
unusual, neurological complications such as
stroke, amaurosis fugax, transient cerebral
ischemia, syncope, and coma are possible.
Neurological signs and symptoms indicate the
need for further diagnostic testing. Surgical
resection is the treatment of choice.
-
- CAROTID ARTERY DISSECTION
-
- Although rare, carotid dissection is a
leading cause of stroke in children and young
adults. It typically presents with the acute
onset of cervical, facial, or head pain,
followed within hours or days by ischemic
symptoms. Biousse and colleagues reported a
posttraumatic carotid dissection presenting with
carotidynia as its sole manifestation. Symptoms
also may include Homer's syndrome, pulsatile
tinnitus, or amaurosis fugax. The artery may
recanalize with time. Carotid duplex
ultrasonography, magnetic resonance imaging
(MM), and carotid arteriography are the
diagnostic tests of choice. The usual treatment
is intravenous heparin followed by 6 months of
warfarin therapy. Surgical intervention may be
required in cases involving increasingly
frequent or severe ischemic symptoms or other
signs of neurological deterioration.
-
- CAROTID ARTERY OCCLUSION
-
- Because of the efficacy of the collaterals
of Willis, total occlusion of one carotid artery
may be neurologically silent. Donnan and
Bladin'4 described two cases of carotidynia
caused by incomplete obstruction of the internai
carotid artery by long intraluminal clots
originating from subintimal hemorrhages beneath
atheromatous plaques. The symptom of carotidynia
was attributed to local vascular trauma. Both
patients presented with neurological symptoms,
as in all of the reported cases of carotid
aneurysm with or without dissection. Clearly,
the presence of ischemic or neurological signs
or symptoms indicates the need for color duplex
Doppler, MRI, or arteriogram.
-
-
- Conclusions
-
- Carotidynia is a painful symptom rather than
a disease. Proper management of this condition
depends on the underlying cause. The most
important step to take when a patient presents
with unilateral neck pain is to make a positive
diagnosis. In the case of carotidynia, this
involves eliciting a history of medical
conditions, such as migraine or upper
respiratory tract infection, and other symptoms
(especially neurological or ischemic). A careful
examinalion including oral and dental
examination, neck palpation, and possibly
indirect or fiberoptic laryngoscopy, is
mandatory.
-
- If Fay's sign is present, the initial
laboratory evaluation should include a
sedimentation rate. If neurological signs or
symptoms are present, a duplex color Doppler,
and depending on the result as well as the
presentation of the patient, either MM or
arteriography should be performed. In most
cases, the sedimentation rate and neurological
history and examination will be normal. In these
cases, a diagnosis of benign carotidynia can be
made.
-
- Patients with a history of migraine and a
prolonged but mild course of carotidynia may be
treated with appropriate migraine therapy.
Younger patients with a more acute course, no
history of migraine, and symptoms suggesting
viral infection may be treated symptomatically
without further evaluation other than a
sedimentation rate. Most of these cases,
especially those with an underlying viral
etiology, will respond to heat, reassurance,
antiinflammatory agents, and time. Reassurance
is important because unilateral neck pain of
some duration is often sufficient to cause a
great deal of anxiety in some patients and their
physicians because of its unknown nature and the
fear of cancer.
-
- The ultimate treatment in severe and
recalcitrant cases may be denervation of the
carotid bulb. In a case reported by de Vries and
colleagues,'9 the pain was so severe that the
patient had been unable to eat, and medical
management had failed. Surgical denervation
resulted in immediate and lasting relief. Fay
also recommended stripping of the common carotid
artery and bulb in refractory cases. This
obviously represents a radical approach that
should be undertaken only in rare cases with no
other option.
-
- Superior
laryngeal neuralgia: carotidynia or just another
pain in the neck?
- O'Neill B, Aronson A, Pearson B, Nauss
L
- Headache
- 1982;22:6-9
-
- The
myth of carotidynia
- Biousse V, Bousser M.
- Neurology
- 1994;44:993-995
-
- Carotidynia:
a pain syndrome
- Hill LM, Hastings G
- J Fam Pract
- 1994;39:71 -75
-
- Fay T. Atypical facial neuralgia. Arch
Neurol Psychiat.1927;18:309-315
- Fay T.
Atypical facial neuralgia, a syndrome of
vascular pain. Ann Otol Rhinol Laryngol 1932;
41; 1030-1062
- Buetow
MP, Delano MC. Carotidynia. AJR Am J
Roentgenol. 2001;177(4):947
- Burton
BS, Syms MJ, Petermann GW, Burgess LPA. MR
imaging of patients with carotidynia. AJNR
2000;21:766 -769
- Arning
C. Ultrasonography of Carotidynia. Am. J.
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