Abstract Eagle’s syndrome (ES) is noticed when there

 Abstract

Eagle’s
syndrome (ES) is noticed when there is an elongated styloid process or
calcified stylohyoid ligament which causes recurrent throat pain or foreign
body sensation, dysphagia, or facial pain. It is not usually reported but it is
more prevalent than generally considered. 
 In adults
the length of the styloid process is approximately 25 mm with a tip located
between the external and internal carotid arteries, lateral to the pharyngeal
wall and the tonsillar fossa. Ossification of the stylohyoid and
stylomandibular ligament leads to prolongation of the styloid process causing
clinical symptoms.  It is diagnosed
radiologically as well as by physical examination. Advanced radiological
modality like CBCT is very valuable in knowing the exact length and extent of
the styloid process. The treatment is primarily surgical. The
physician’s knowledge of possible clinical variations and its diverse
symptomatology is critical.

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Keywords:
Eagle’s syndrome; Stylohyoid syndrome; Elongation; Styloid process.

 

Introduction           

Watt W.
Eagle was the first to describe the clinical findings of eagles syndrome in the
year 1937.1 Eagles
syndrome or stylohyoid syndrome, is the symptomatic elongation of the styloid
process or mineralization of the stylohyoid ligament complex. The styloid
process is a bony projection, which is situated immediately anterior to the
stylomastoid foramen. It is of cylindrical form which projects downwards from
the inferior surface of the temporal bone towards the front, downwards and
medially narrowing at the tip. The position of the tip is important, as it is
situated between the internal and external carotid arteries, laterally from the
pharyngeal wall and just behind the tonsil fossa. Two ligaments and three
muscles are attached to the styloid process. Stylopharyngeus attaches medially
and from the posterior side close to the base of the process, stylohyoid from
the posterior side and laterally on the central part of the process and
styloglossus starting from the anterior part of the process is seen next to the
tip. The muscles are supplied by glossopharyngeal, facial and hypoglossal
nerve. The stylohyoid ligament extends from the tip of the styloid process up
to the lesser cornu of the hyoid bone and the stylomandibular ligament,
commences under the attachment of styloglossus muscle and ends near the angle
of mandible.2 ES
is an uncommon entity which is not always suspected in daily practice. Only
about 4% of the population is believed to have an elongated styloid
process.  Patients with ES presents with
a sore throat, ear pain, or even with symptoms of foreign Body in the pharynx
secondary to pharyngeal and cervical nerve interactions. Symptoms are common in
females. Patients are usually above 30 years. As the symptoms are variable and
non-specific, patients seek treatment in several different clinics.3
We, here report a case of eagles syndrome who presented with neck pain.

 

 

 

Case history

A
44 year old male patient reported to our OPD, with a chief complaint of pain in
left side of the ear and neck since 1 year (Figure 1).  History revealed that the pain was
unilateral, insidious in origin, dull to moderate in intensity and intermittent
in nature. The pain was characterized by needle-like, piercing pain inside the
left ear, pain was associated with headache. The intensity of pain was
aggravated by movements such as looking up and turning face to right side and
became worse during deglutition. In addition, patient also had a sensation of
foreign body in throat while swallowing. There was no relevant medical or
dental history.  A patient was moderately
built and nourished, and mentally sounds. On extraoral examination,
temporomandibular joint showed bilaterally symmetrical movements, clicking was
present on left side, along with tenderness in the pre-auricular region. Also
tenderness between upper end of sternocleidomastoid muscle and angle of
mandible. A small bony pointed projection was palpable in the right
submandibular region at the anterior border of sternocleidomastoid muscle,
which was tender. Mouth opening was 32mm and mandibular protrusion was 10mm. On
intraoral examination, he had a normal complement of teeth, with no carious or
filled teeth, and with moderate stains and calculus. The medial pterygoid
muscle was tender on palpation on left side. Based on the history and clinical
findings, a provisional diagnosis of Myofacial Pain Dysfunction Syndrome was
put forth. And Eagles syndrome was considered under differential diagnosis.
Panoramic radiograph showed that the length of left styloid process to be 48mm,
with Langlais type I pattern (Figure 2). Considering the elongated styloid
process and suspecting eagles syndrome, CBCT was advised. CBCT scan showed
unilateral elongation of the styloid process towards the left. The actual
length of the styloid process was processed using CBCT in various sections
(Figure 3, A-D), and was found to be 62.2mm in comparison with the OPG, which
was 48 mm. Almost a difference of 14 mm was noticed between the two modalities.
Hence showing, CBCT as the most reliable radiologic modality, to be carried out
in cases where styloid process elongation is suspected. Based on the history,
clinical findings and investigations, a final diagnosis of Eagles Syndrome was
given and the patient was referred, for the removal of the styloid process.

 

Discussion

Stylos refers to pillar derived from a
Greek word. Styloid process, stylohyoid ligament and small horn of the hyoid
bone developmentally originate from the second branchial or hyoid arch. The
elongation of styloid process can be congenital or calcification of
stylohyoid/stylomandibular ligament as a result of ageing and other
degenerative process. Because of its cartilaginous origin, the ligament has the
potential to mineralize. There exists difference between true Styloid Process
elongation and secondary ossification of the stylohyoid ligament. True
elongation results in a smooth, regular, well corticated bone of different
lengths projecting continuously from the skull base as seen in our case.
Secondary stylohyoid ligament ossification results in an irregular surface with
thickened areas that extend towards  the
lesser horn of the hyoid bone, usually with prominent medial angulations. The
ossified complex may or may not be be segmented with a thin cortex or a bulky
irregular contour.4 Other names include “Elongated Styloid Process
Syndrome”, “Carotid Artery Syndrome”, “Styloid Process Neuralgia”, “Stilalgia”,
“Stylohyoid Syndrome” and “Pseudo hyoid Syndrome”.5 Eagle’s syndrome
must always be considered for the differential diagnosis of pains localized in
the head-neck area, especially in persons above the age of 30 years. It is an
uncommon but one of the main cause for chronic head and neck pain. The characteristic dull and nagging pain which becomes bad during
deglutition and can be felt during palpation of the tonsillar fossa is the
hallmark sign.3
Elongated styloid process may lead to compression of many vital structures and
cause inflammatory changes like chronic pain in the pharyngeal region,
radiating otalgia, phantom foreign body sensation (globus hystericus), pain in
the pharyngeal region, dysphagia etc,. And also can cause craniofacial as well
as cervical pain, difficulties while swallowing, secondary glossopharyngeal
neuralgia, radiating pain into orbit and maxillary region.6

 

Vaious pathophysiological mechanisms for the
pain of ES includes7

·                    
Compression of the neural
elements, the glossopharyngeal nerve, ,the chorda tympani by the elongated
styloid process and the lower branch of the trigeminal nerve;

·                    
Fracture of the ossified
stylohyoid ligament, is usually followed by proliferation of granulation tissue
causing pressure on surrounding structures and results in pain;

·                    
Impingement on the carotid
vessels by the styloid process, causing irritation of the sympathetic nerves in
the arterial sheath;

·                    
Degenerative and
inflammatory changes in the tendonous portion of the stylohyoid insertion;

·                    
Irritation of the
pharyngeal mucosa by direct compression of the styloid process;

·                    
Stretching and fibrosis of
the fifth, seventh, ninth, and tenth cranial nerves in the post-tonsillectomy
period.

 

Langlais
et al, in the year 1986 put forth a radiological classification for elongated
styloid process8

·                    
Type I (Elongated styloid
process) pattern that represents an uninterrupted, elongated styloid process,
which is reported in our case.

·                    
Type II (Pseudoarticulated
styloid process) is characterized by the styloid process being joined to the
stylohyoid ligament by a single pseudo articulation which gives the appearance
of an articulated elongated styloid process.

·                    
Type III (Segmented styloid
process) consists of interrupted segments of the mineralized ligament, which
gives the appearance of multiple pseudo articulations within the ligament.

 

Differential
diagnosis for eagles syndrome includes laryngopharyngeal dysesthesia, temporomandibular
arthritis, glossopharyngeal and trigeminal neuralgia, chronic
tonsillo-pharyngitis, Sluders syndrome, cluster type headache, temporal
arteritis, cervical vertebra arthritis, benign or malignant neoplasms,
migraine, tension headache, atypical facial pain, myofascial pain syndrome,
tonsillitis, psychosomatic disease, trigeminal neuralgia, TMJ disorders,
temporal arteritis, unerupted or impacted molar teeth and faulty dental
prostheses, costens syndrome and trotters syndrome.9

The
most reliable radiological investigation in recent times is the CBCT, where the
exact length and extent of the styloid process can be studied. This syndrome
may be treated conservatively or surgically. Conservative treatment include
transpharyngeal injection of steroids with lignocaine, nonsteroidal anti-inflammatory
drugs, diazepam, application of heat, traditional chinese medicines, and
transpharyngeal manipulation by manually fracturing the styloid process. The
most satisfactory, effective and followed treatment is surgical shortening of
the styloid process, either through an intraoral or external approach.
Advantage of an external approach is the proper exposure of the styloid process
and the adjacent structures, and this advantage outweighs all the other considerations
and also facilitates the resection of a partially ossified stylohyoid ligament.
Transoral resection prevents outside scars, but carries the risk of deep
cervical infection with possible injury to the neurovascular bundles.10

 

Conclusion

The elongated styloid process syndrome
can be easily diagnosed by a detailed history, physical examination, and
radiological investigations. It should always be taken to consideration in patients with a
sore throat and pain while 
swallowing,  feeling of foreign
body in the throat and facial pain. Clinicians should 
consider the possibility of Eagle’s syndrome when both the clinical and
radiographic evidence are positive. Every practitioner should be aware
of it to prevent misdiagnosis and unnecessary trouble to the patient.

 

 

References

1.                  
Sadaksharam
J, Singh K. Stylocarotid syndrome: An unusual case report. Contemp clin Dent
2012; 3:503-6.

2.                  
Teki
S, Ashalatha D, Babu S, Kumari KL, 
Prasad S, Vasanthi A. Eagle’s
Syndrome- Elongated Styloid Process. IOSR-JDMS 2014;13(5):31-33.

3.                  
Khandelwal
S, Hada YS, Harsh A.Eagle’s syndrome – A case report and review of the
literature. The Saudi Dental Journal 2011;23:211–5.

4.                  
Pushpalatha
K, Bhat D. Bilaterally Elongated Styloid Process – A Case Report. Int J Sci
Stud 2014;2(4):100-102.

5.                  
The
Internet Journal of Biological Anthropology Generated at: Wed,03 Jul 2013.
(Accessed on 07 July 2013).

6.                  
Kosar
MI, Atalar MH, Sabancio?ullari V, Tetiker H, Erdil FH, Cimen M, Ota? I.
Evaluation of the length and angulation of the styloid process in the patient
with pre-diagnosis of Eagle syndrome. Folia Morphol 2011; 70(4):295-9.

7.                  
Ceylan, A., Ko ybas iog lu, A., Celenk,
F., Yilmaz, O., Uslu, S. Surgical treatment of elongated styloid process:
experience of 61 cases. Skull Base 2008;18(5):289–295.

8.                  
Chauhan
S, Mohan V, Chauhan R. Bilateral Elongated Styloid process- A Case Report. J
Dent Specialities 2016;4(1):82-86.

9.                  
Khandelwal
SA, HadaYS. Eagles syndrome-A
case report and review of the literature. Saudi Dent J 2011;23(4):211-15.

10.               
Uluda? IF,
Öcek L. Eagle syndrome: case report. Agri 2013;25(2):87-89.

 

Figures

 

 

Figure
1: Patient,
suffering from neck pain.

 

 

Figure
2: OPG of
the patient revealing elongated left styloid process, with measurement.

 

 

 

 

 

Figure
3: CBCT
revealing elongation of left styloid process, in various sections. A & B Coronal
and sagittal section showing elongation of left styloid process.

C-Coronal section showing right styloid
process, whose length is under normal limits.

 

 

D-3D view of the elongated left styloid
process.