94 EAGLE’S SYNDROME IN AN 11-YEAR-OLD PATIENT

J Oral Maxillofac Surg

59:94-97, 2001

Eagle’s Syndrome in an
11-Year-Old Patient
Faisal A. Quereshy, DDS, MD,* Evan S. Gold, DMD,†
Jim Arnold, MD,‡ and Michael P. Powers, DDS, MS§

In 1937, W.W. Eagle documented cases in which
elongation of the styloid process (over 25 cm), or
Received from the University Hospitals of Cleveland; Case Western
Reserve University, Cleveland, OH.
* Assistant Professor, Department of Oral and Maxillofacial Surgery.
† Resident, Department of Oral & Maxillofacial Surgery.
‡ Chairman, Department of Otolaryngology/Head and Neck Surgery.
§ Chairman, Department of Oral & Maxillofacial Surgery.
Address correspondence and reprint requests to Dr Quereshy:
2123 Abington Rd, Case Western Reserve University, Department
of Oral and Maxillofacial Surgery, Cleveland, OH 44106.
© 2001 American Association of Oral and Maxillofacial Surgeons
0278-2391/01/5901-0018$3.00/0
doi:10.1053/joms.2001.19302
calcification of the stylohyoid ligament, appeared to
be the cause of pharyngeal and cervical pain.1 The
prevalence of an elongated styloid process is some-
what varied, although Fritz reports it as a common
finding.2 Eagle believed that approximately 4% of the
population had an elongated styloid process, and that
only 4% of these persons showed symptoms.3 Kauf-
man et al4 radiographically found a 7.3% incidence of
elongation in their sample, but no correlation to clin-
ical symptoms was noted. Most cases are found in
women, with an age distribution greater than 30 years
of age.1,5,6 There are no data on the prevalence of an
elongated styloid process in children.
Eagle’s syndrome comprises symptoms that may
include a dull, acing pain localized in either or both

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QUERESHY ET AL
sides of the throat, with or without referred pain to
the ear and mastoid region on the affected side. Some
patients may complain of pain on swallowing (dys-
phagia) or an abnormal sensation of a foreign body in
the pharynx (globus hystericus).7 Other symptoms
that aid in the diagnosis include pain with rotation of
the head, recurrent headache, and vertigo.8
The purpose of this report is to describe a pediatric
patient with the diagnosis of Eagle’s syndrome and to
review the proposed treatment options.
Report of Case
An 11-year-old girl presented to the Department of Oral
and Maxillofacial Surgery at Case Western Reserve Univer-
sity/University Hospitals of Cleveland, with a chief symp-
tom of pain in the right neck that worsened on turning the
head to the right. The dull, aching pain radiated to the right
preauricular and postauricular areas. The patient also re-
ported intermittent dysphagia, nonspecific for certain food
types. Her medical history was negative for any coexisting
diseases, and there was no family history of craniofacial
syndromes.
On physical examination, tenderness to palpation was
elicited bilaterally in the tonsillar fossa region. She had a
limited range of neck motion, especially rotation. Neck
masses were neither palpable intraorally nor externally, and
there was no cervical or submandibular lymphadenopathy.
The remainder of the neck examination was normal, with a
nonpalpable thyroid and a midline trachea.
Radiographic evaluation consisted of a panoramic radio-
graph that showed bilateral radiopaque bodies extending
from the styloid processes lateral to the mandible (Fig 1). A
FIGURE 1. Initial panoramic radiographic showing the elongated
right stylohyoid process posterior to the mandibular ramus (arrow). A
similar process was identified on the left side.
95
confirmatory lateral cephalometric radiograph was ob-
tained. laboratory blood workup consisted of a SMA-23,
erythrocyte sedimentation rate, and complete blood cell
count, which were all within normal values. A presumptive
diagnosis of Eagle’s syndrome was made, and conservative
therapy was initiated for a period of 4 weeks. Because the
heat applications, analgesics, and rest did not improve the
patient’s symptoms, the option of surgical intervention was
discussed. Furthermore, even though the patient’s symp-
toms were predominately on her right side, similar findings
on the left side warranted prophylatic surgical removal of
that styloid process. A preoperative 3-dimensional com-
puted tomographic scan of the head and neck region was
obtained for evaluation of the anatomic proximity of the
calcified styloid processes to the adjacent vasculature and
other vital neck structures (Fig 2).
After the intravenous administration of prophylactic an-
tibiotics and obtaining general anesthesia, the patient’s
neck was placed in a hyperextended position, and her head
was rotated away from the surgeon. A skin incision was
made several centimeters below the right mandibular ramus
and inferior border. The anterior border of the sternoclei-
domastoid muscle was exposed, as were the stylohyoid
muscle and the posterior belly of the digastric muscle over-
lying the carotid artery, which were retracted posteroinfe-
riorly. Care was taken to identify the hypoglossal nerve,
confirmed by nerve stimulation, and it was retracted ante-
riosuperiorly out of the surgical field. The anterior fibers of
the elongated, calcified stylohyoid ligament were noted to
be loosely connected to the greater cornu of the hyoid
bones bilaterally. These fibers were transected by using a
tenotomy scissors, and the ligament was followed postero-
superiorly to the base of skull by using a “ring curette” and
Freer elevator (Fig 3). The styloid process was then short-
ened 50 mm by using a side-biting Kerrison rongeur. A
similar procedure was then done on the left side, and 39
mm of the process was removed (Fig 4). The specimens
were sent for mineral content analysis, which showed pre-
dominately calcium and phospate. Postoperatively, the pa-
tient had complete relief of symptoms. There was no evi-
dence of hypoglossal or facial nerve involvement. The
patient has remained pain free after 12 months of follow-up.
Postoperative radiographs have not shown evidence of re-
currence.
Discussion
The cause of an elongated styloid process or calci-
fication of the stylohyoid ligament is unclear. Several
earlier reports have attempted to hypothesize its oc-
currence. One such mechanism describes a congeni-
tal elongation of the styloid process attributable to the
persistence of a cartilaginous element that connects it
to the temporal bone, which may grow abnormally
and be converted to bone.9 Research has provided
histologic evidence for metaplastic changes in the
subperiosteal cells in the vicinity of the stylohyoid
ligament insertion that ultimately results in the
growth of the osseous tissues.9-11 Yet another pro-
posed mechanism is the elongation or extension of
the osseous styloid process so that it merges with an
independently calcified stylohyoid ligament.9-11
96
FIGURE 2. Three-dimensional computed tomographic scan showing
the precise location of the calcified stylohyoid ligaments. Note the
bilateral extension to the greater cornu of the hyoid bone.
The different pain mechanisms also have been stud-
ied. With sudden jerking of the head, fracture of the
ossified stylohyoid ligament can occur, and a non-
union may develop because of continuous micro-
movement of the hyoid bone. The proliferation of
granulation tissue in the region of the nonunion could
then cause pressure on the surrounding structures,
resulting in pain in that area.12 Compression of neural
elements by the elongated styloid process has also
been proposed as a pain mechanism, with the glosso-
pharyngeal nerve, lower branch of the trigeminal
nerve, or the chorda tympani being directly in-
volved.13 The tendinous region of the stylohyoid lig-
ament insertion also may show degenerative or in-
flammatory changes resulting in a tendinosis as a
cause of the pain. This phenomena is seen also in
other narrow-based muscle tendon insertions that are
anchored directly to bone.11 The pharyngeal mucosa
FIGURE 3. Intraoperative view of the elongated osseous extension of
the stylohyoid ligament. After identification and retraction of the stylo-
hyoid and posterior digastric muscles, as well as the submandibular
gland superoanteriorly, the styloid process can be visualized. A ring
curette was used to free the soft tissue attachments along the entire
length of the process.
EAGLE’S SYNDROME IN AN 11-YEAR-OLD PATIENT
FIGURE 4. Calcified styloid process and stylohyoid ligament after
removal. Note the impressive lengths: left, 39 mm; right, 50 mm.
also may become chronically irritated and painful as
continuous stretching of the mucosa over the pointed
styloid process occurs. Pain also may be in the form of
dysesthesia, where fibrosis results from post-tonsillec-
tomy healing of the sensory nerve endings (cranial
nerves V, VII, IX, and X), all of which have branches
that supply the involved area.14 Lastly, impingement
on the carotid vessels by the styloid process, affecting
the circulation and producing irritation of the sympa-
thetic nerves in the arterial sheath, has been sug-
gested as a cause of the dull, aching pain seen in these
patients.
There are numerous conditions to consider in the
differential diagnosis of neck pain. Cranial nerve neu-
ralgia, such as that involving the trigeminal, glosso-
pharyngeal, superior laryngeal, and primary genicu-
late ganglion,10 temporomandibular joint disease,1,10
chronic pharyngotonsilitis,13 unerupted or impacted
molar teeth,1,5 improperly fitting dental prostheses,13
and pharyngeal and tongue base tumors13 all may
cause referred pain similar to that produced by an
elongated stylohyoid process. Neck pain in the pedi-
atric patient also must warrant an appropriate evalu-
ation for any evidence of neck masses, including con-
genital cysts, such as the branchial cyst, teratoma, or
dermoid cyst, and neoplastic processes of benign or
malignant origin, such as a lipoma, fibroma, liposar-
coma, and fibrosarcoma.15,16
The description of a constant, dull pain worsening
during swallowing, palpation of the tonsillar fossa and
retromandibular area eliciting pharyngeal pain, and a
radiologic demonstration of an elongated styloid pro-
cess should alert the clinician to a possible diagnosis
of Eagle’s syndrome. To clinically localize the site of
the pain, a diagnostic local anesthetic block can be
administered in the tonsillar fossa region. Relief of the
pain (a positive test) would provide the clinician with
a basis to consider a possible diagnosis of Eagle’s
QUERESHY ET AL
syndrome. The diagnosis can be further confirmed
with plain radiographs such as the lateral skull film,
posteroanterior skull film, oblique mandible films,
and Towne’s or panoramic views. Computed tomog-
raphy (CT) is useful to study the anatomic relation-
ships between the elongated bony process and the
adjacent vital vascular structures. It is very difficult to
ascertain whether the styloid process is elongated or
whether the stylohyoid ligament is calcified from ra-
diographic findings alone. Both processes produce
radiodensities on plain film radiography and hyper-
densities on CT scanning. Clinically, it is difficult to
distinguish which of the 2 entities exist, or if there is
a coexistence. However, analyzing the specimen for
its mineral content may help to distinguish an elon-
gated process from a calcified ligament.
The only satisfactory and effective treatment to
completely eliminate symptoms caused by an elon-
gated or misdirected styloid process is surgical
shortening.9 There are well-accepted intraoral and
extraoral surgical approaches. Intraorally, access is
relatively easy, and is generally a less time-consum-
ing procedure than the extraoral operation,9 with a
shorter recovery period.17 It can be performed un-
der local anesthesia, avoiding the morbidity and
mortality associated with a general anesthesia. The
incision is made in the tonsillar fossa, so unsightly
external scars are be avoided.17 However, this ap-
proach is not without risk, with the possibility of a
deep cervical infection associated with bacterial
contamination after opening of the pharynx into
the neck. Also, the posterior pharynx is a poorly
visualized region and, therefore, has the increased
risk of trauma to the external carotid artery or to
the cranial nerves V, VII, IX, X, and XI that course
in the surgical field.17
The extraoral approach offers the advantage of bet-
ter exposure, especially of the bifurcation of the ex-
ternal carotid artery and the styloid process. There is
also decreased risk of bacterial contamination associ-
ated with a properly prepared sterile surgical field.9
The disadvantages include the necessity of general
anesthesia and endotracheal intubation, with its in-
97
herent risks and complications. Also, with today’s
society focused on aesthetics, creating a scar on the
neck is undesirable.9
The exact cause of Eagle’s syndrome is still consid-
ered a mystery. Most patients with calcified stylohy-
oid processes are asymptomatic. Conversely, many
patients with the constellation of vague symptoms of
neck, ear, and pharyngeal discomfort do not show
radiographic evidence of an elongated/calcified stylo-
hyoid process. However, despite the age of the pa-
tient, clinicians should consider the possibility of
Eagle’s syndrome when both the clinical and radio-
graphic evidence support the diagnosis.
References
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two cases of styloid process– carotid artery syndrome with
operation. Arch Otolaryngol 49:490, 1949
2. Fritz M: Elongated styloid process. Arch Otolaryngol 31:911,
1940
3. Eagle WW: Elongated styloid process. Arch Otolaryngol 67:172,
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4. Kaufman SM, Elzay RP, Irish EF: Styloid process variation. Arch
Otolaryngol 91:460, 1970
5. Eagle WW: Elongated styloid process: Report of two cases.
Arch Otolaryngol 25:584, 1937
6. Schmidt OVM: Elongated styloid process which interfered with
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