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Interventional Pain Management Reports
ISSN 2575-9841 Volume 2, Number 4, pp 133-139
2018, American Society of Interventional Pain Physicians©
Case Report
From : Henry Ford Health System, Detroit, MI
Author for correspondence: Gaurav Chauhan, MD
Address: Department of Anesthesiology, Henry Ford Health System,
Detroit, 1350 W. Bethune St., Apt 1607, Detroit, MI 48202
E-mail: Gchauha1@hfhs.org
133
EaglE SyndromE: a diagnoStic challEngE with a novEl Solution
Gaurav Chauhan, MD, Sandeep Kataria, MD, Jie-Xin Tang, MD, Vivek Loomba, MD, and
Aman Upadhyay, MD
with sudden onset of pain, 3 years ago, in the right jaw
with radiation to the right part of neck and entire face.
At that point, he received emergency treatment for
myocardial infarction but subsequent investigations
including cardiac enzymes, electrocardiogram and
stress echocardiogram came back negative for any
acute cardiac pathology.
During the course of the next year, he presented
multiple times to the emergency department and at
his general practitioner’s office with similar kinds of
pain. He also reported a waxing and waning course
of pain with severity of pain ranging from 2-8/10 on
the numeric rating scale. Over the course of 1 year
he was assigned various diagnoses such as dental
malocclusion, chronic otitis media, eustachian tube
dysfunction, otalgia associated with chronic neck
pain, etc., and received treatment ranging from a
short-term course of antibiotics and opioids along with
various non-steroidal anti-inflammatory (NSAIDs)
drugs.
He was finally referred to the otolaryngology clinic
where he was diagnosed with temporomandibular
joint (TMJ) dysfunction along with myofascial pain
syndrome. He was prescribed Gabapentin 300 mg 3
times a day, Topiramate 80 mg twice a day, Methocar-
bamol 750 mg 3 times a day, Diclofenac 50 mg twice
a day and Desipramine 10 mg nightly. Patient was
also given a referral for physical therapy to stretch
and strengthen his neck and jaw muscles.
The patient reported vivid dreams and unpleasant
sensations after starting Gabapentin therapy. He
was immediately advised to stop Gabapentin, which
was substituted for Carbamazepine. The physi-
cian was unable to prescribe Pregabalin as it was
not prescribed, and as it was not covered by the
patient’s health insurance. Over the course of a few
Eagle Syndrome (ES), also referred to as styloid or
stylohyoid syndrome, was first reported by otolaryn-
gologist Watt W. Eagle in 1937. It refers to the myriad
of signs and symptoms associated with an elongated
styloid process or calcified stylohyoid ligament com-
plex compressing on adjacent anatomical structures
(1). The clinical spectrum of ES is comprised of non-
specific presentations such as facial pain, sore throat,
otalgia, dysphagia, and or headaches (2). Patients
suffering from ES are assigned various differential
diagnosis and they may seek treatment at several dif-
ferent clinics such as otolaryngology, family practice,
neurology, neurosurgery, psychiatry, or dentistry (3).
We present a case report of a patient suffering from
ES, who was referred to the chronic pain clinic as a
last resort from the otolaryngology clinic. The chronic
pain team performed a successful fluoroscopic-
guided injection consisting of the steroid and local
anesthetic mixture at the tip of the styloid process,
leading to immediate and profound pain relief. This
manuscript discusses the unique pathomechanics,
diagnostic challenges and the solution offered by
the authors.
caSE rEPort
We present a case report of a 57-year-old male, who
consented to this case report to be published. The
subject reported pain in right neck since last 3 years
and sought multiple consults and assessments over
the years with general practitioner, surgeon, dentist,
and even a psychiatrist. The patient first presented
IPM Reports
134
IPM Reports Vol. 2, No. 4, 2018
weeks Desipramine was also discontinued, due to
complaints of oral dryness and was substituted with
Nortriptyline 25 mg nightly. At 3 months follow-up,
the patient reported that conservative management
and physical therapy was successful in offering a
mild decrease in the discomfort due to chronic pain
but was not useful during the episodes of acute pain.
The otolaryngology team ruled out ES from the dif-
ferential and the patient further underwent treatment
for multitude of differentials including sinus infection,
sensorineural hearing loss, neuropathic pain, etc.
without any significant relief. At 3 years from the
initial presentation and at 2 years from presentation
at otolaryngologist’s office, after multiple cycles of
diagnostic and therapeutic failures, patient reported
that he was getting frustrated and was beginning to
feel depressed due to his chronic pain.
He further reported that the pain was adversely
affecting his quality of life. In lieu of his suboptimal
symptom control he was referred to the pain clinic and
to a chronic pain psychologist. In the pain clinic, on
examination, the patient reported no change in char-
acter of his right-sided, anterolateral and upper neck
pain which was exacerbated by yawning or turning
the head on the opposite side and was associated
with dysphagia, odynophagia and globus sensation
in the throat. He did not endorse any trauma, nor
had he any history of a procedure or surgery on the
right side of the neck. There were no obvious bony
processes observed or perceived on palpation of the
neck but patient-reported pain on palpation of the right
peritonsillar fossa.
The patient had been taking Diclofenac 50 mg twice
a day, Methocarbamol 750 mg 3 times a day, along
with Nortriptyline 25 mg nightly at this point, which
seemed to offer some degree of benefit. The lateral
x-ray of neck and computerized tomographic (CT)
scan revealed a bilateral elongated styloid process
(left side - 6 cm and right side - 5 cm) which was
more pronounced on the asymptomatic side (left)
(Figs. 1 and 2).
On the basis of the physical exam, radiographic
evidence of elongated styloid processes, protracted
clinical course and vague symptomatology, a possibil-
ity of ES was reconsidered, primarily as a diagnosis
of exclusion. Consequently, the authors planned to
perform a fluoroscopically-guided injection of a mix-
ture of local anesthetic and non-particulate steroid at
the tip of the styloid process on the affected side as
a diagnostic and therapeutic option.
The risks, benefits and alternatives were explained
in detail to the patient and he consented to the pro-
cedure. The right-sided styloid process was identified
by fluoroscopy in the lateral projection. For added
safety, the approximate depth of the styloid process
was extrapolated from the previous CT images. Un-
der aseptic precautions, using a 25 gauge, 1½-inch
needle, the right-sided styloid process was reached
in fluoroscopic projection view and was confirmed
with the anteroposterior view. A 0.5 mL iodinated
contrast was injected to confirm position and rule
out the abnormal spread (Fig.3). A 3 mL solution
comprising 2 mL of 0.5% Bupivacaine and 4 mg of
Dexamethasone, was injected in the intended space.
The patient reported excellent and an almost im-
mediate relief of his pain. The pain relief lasted for 9
days and the patient further commented that this was
the only thing that had helped his disabling condition
in the past 3 years.
These new findings were communicated to the oto-
laryngology team and the patient was subsequently
scheduled for excision of the right styloid process via
an extra-oral approach. During the surgical procedure
a solution of 1% lidocaine with 1:200000 epinephrine
was injected into the left neck 2 fingerbreadths below
the angle of the mandible.
A standard neck incision was made 2 fingerbreadths
below the angle of the mandible from mastoid to the
edge of the mandible. The dissection was performed
along the sternocleidomastoid muscle (SCM). The
posterior auricular nerve was identified and spared
and the anterior border of the SCM was dissected free
from underlying soft tissue. A blunt dissection was then
used to identify the spinal accessory nerve and this was
dissected free from the overlying tissue. The posterior
belly of the digastric was identified and dissection was
then performed medially deep to the digastric muscle.
The stylomastoid muscle was identified and dissected
off at the styloid process at the angle of the mandible
Eagle Syndrome: A Diagnostic Challenge with a Novel Solution
135
IPM Reports Vol. 2, No. 4, 2018
Fig. 1. CT Scan of Cervical soft tissue - Sagittal view, serial views from right to left. Yellow arrow depicting the sub-
segmental ossification of both styloid processes. The ossified ligaments run all the way to the lesser cornua of the
hyoid bone bilaterally, indenting the base of the tongue, right vallecular space, and running in between the external
and internal carotid arteries. The right demonstrates more ossification and calcification compared to the left. There is
no clear-cut attachment to the temporal bone of stylohyoid ligament.
using a freer elevator and a periosteal elevator. The
styloid process was then stripped of the tissues in all
directions. A bone cutter was then used to cut the distal
portion of the styloid process. The bone was removed. A
rongeur was used to round the proximal cut edge of the
styloid process. The incision was then re-approximated
with 3-0 vicryl and the superficial skin was closed
with DERMABOND adhesive (Ethicon US, LLC). The
patient reported excellent pain relief with remission of
symptoms at 6 months follow-up after the surgery. He
further reported that he has stopped taking Diclofenac
and requested for Nortriptyline to be tapered off.
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Fig. 2. Lateral x-ray of cervical region: Bilateral segmental ossification of the stylohyoid processes and ligaments.
Right styloid is more ossified than the left styloid process.
diScuSSion
The embryonic origin of the styloid process is from
endochondral ossification of the Reichert’s cartilage
of the second pharyngeal arch (4). It is an elongated,
conical, variably tapered extension of the inferior
surface (petrous part) of the temporal bone that
projects downwards and forwards from the inferior
surface of the temporal bone. The styloid process
lies between the internal and external carotid arteries
and is bounded posteriorly by the mastoid process
and medially by tonsillar fossa. Its proximal part,
known as tympanohyal is encased by the tympanic
aspect of the temporal bone and its distal part, also
known as stylohyal, serves as an insertion point
for the stylohyoid and stylomandibular ligaments
and styloglossus, stylohyoid and stylopharyngeus
muscles. The stylohyoid ligament connects the
apex of the styloid process and the lesser horn of
the hyoid bone, and the stylomandibular ligament
extends from the styloid process to the parotideo-
masseteric fascia between the mastoid process and
the mandible (2,5).
Eagle Syndrome: A Diagnostic Challenge with a Novel Solution
137
IPM Reports Vol. 2, No. 4, 2018
Fig. 3. A sagittal fluoroscopic view of base of skull and neck depicting the tip of styloid process localized by injection
of Isovue contrast dye.
The length of a typical styloid process ranges from
1.52 to 4.77 cm, however, a 3 cm or longer process is
usually considered anomalous (6). The incidence of an
elongated styloid process and the calcified stylohyoid
ligament is reported to be 3.3% and 18.2%, respectively
but only 1-5% of the subjects with radiographic evidence
develop symptoms (6,7). Due to the lack of epide-
miological studies the current literature is ambivalent
in regards to the sexual predilection of ES; however,
the symptoms are more common in females and the
patients are usually older than 30 years of age (4).
The mechanics of ES may include direct compres-
sion of adjacent structures by an elongated styloid
process due to degenerative and inflammatory
changes in the cartilaginous precursors (insertion
tendinosis) of the stylohyoid ligament (8). The etiol-
ogy of the late onset of symptoms includes reactive
hyperemia, metaplasia due to abnormal healing and
fibrosis following recurrent throat infections, scar tis-
sue formation following trauma (induced or iatrogenic
e.g. tonsillectomy) and age-related changes such
as cervical disc degeneration which may lead to the
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IPM Reports Vol. 2, No. 4, 2018
shortening of cervical vertebrae and altering the direc-
tion of the styloid process which can further cause
distortion of cranial nerves or cranial nerve endings in
structures adjacent to the styloid process (8). Ectopic
ossification or mineralization of the stylohyoid makes
it more vulnerable to fractures. The cycles of recurrent
fractures and subsequent ossification further fuels the
proliferation of granulation tissue, which impinges on
adjacent structures. The constellation of symptoms
in a patient suffering from ES is independent of the
degree of calcification or size of styloid process as
chronic impingement of surrounding tissues in the
pharyngeal mucosa, either directly due to styloid
process or as a result of peri-styloid inflammation,
sets up a cascade of events leading to further chronic
inflammation, edema, granuloma formation, adhe-
sions and fibrosis leading to further irritation and pain.
The neural elements most commonly involved
are the mandibular branch of the trigeminal nerve,
chorda tympani branch of VII nerve, IX and X nerves
(3,6). The diagnosis is guided by the clinical history,
physical exam and corroborated by the radiological
evidence and a high index of suspicion. The charac-
teristic symptoms may include recurrent throat pain
associated with foreign body sensation or dysphagia
along with radiation to ipsilateral ear or jaw and
may be triggered or exacerbated by head rotation,
tongue movement or during deglutition. A careful
transpharyngeal palpation may demonstrate a bony
projection in the tonsillar fossa and may reproduce the
characteristic pain, which may be referred to the ear,
face or head (3,7). The patients frequently complain
of a foreign body sensation in the throat along with
dysphagia, odynophagia, dysgeusia or alterations
in taste and a change in the character of their voice.
Infrequently, patients have also reported migraine-like
headaches and occipital neuralgias as a manifesta-
tion of referred pain (7,9). In more rare instances, an
enlarged styloid process may also impinge upon the
carotid sinus of the internal carotid artery, leading to
the transient ischemic attack, dizziness and vagal
mediated cardiac inhibition, which can prove to be
fatal (10-12).
Various imaging techniques such as panoramic radi-
ography, lateral cephalometry, Towne projection film,
or CT scan, may be employed to aid the diagnosis. A
CT scan or CT guided 3-dimensional reconstruction
can yield the exact spatial orientation, the anatomic
variance of styloid process and its association with the
surrounding tissues (9,10). Despite the radiological
evidence of an elongated styloid process, we used a
mixture of local anesthetic and steroid solution and
injected at the tip of styloid process under fluoroscopic
guidance to further affirm the diagnosis. There are
case reports in which authors have injected local
anesthetic in the tonsillar fossa or employed stellate
ganglion blocks to mitigate pain; however, these
procedures are technically challenging and are not
bereft of serious side effects (13). The fluoroscopic
injection technique is relatively straightforward, and
offers a more precise means of diagnosis as it offers
the advantage of using less of a volume of local
anesthetic injected.
Conservative management should be the first line
of treatment as it has a high success rate (80% of
cases) and can lead to can lead to complete resolu-
tion of symptoms. Conservative management of ES
includes multimodal analgesics such as non-steroidal
anti-inflammatory drugs, which may have added
benefit to reduce local inflammation, gabapentinoids
and tricyclic antidepressants for the neuropathic
components of the pain (14,15). If conservative man-
agement fails or in the presence of acute severity of
symptoms, an en-bloc excision, either extra-oral or
endoscopically guided, of the styloid process offers
definitive management (11). After the dissection of the
right styloid process the subject had gradual decrease
and complete resolution of his chronic throat pain.
In conclusion, the pathognomonic signs and
symptoms of ES are vague and share a multitude
of etiologies making it a diagnostic challenge. In
our case, there was a delay of 3 years from onset
of symptoms to the definitive management, during
which the patient underwent a great deal of stress
and psychological trauma. A multidisciplinary col-
laboration between chronic pain, otolaryngology,
and psychiatry, was required to identify, diagnose
and treat the rare diagnosis of ES. This is the first
instance of the utilization of a simple, fluoroscopically
guided injection technique for confirmatory diagnosis
of this rare condition.
Eagle Syndrome: A Diagnostic Challenge with a Novel Solution
139
IPM Reports Vol. 2, No. 4, 2018
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