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Year : 2013  |  Volume : 4  |  Issue : 2  |  Page : 124-126

Eagle syndrome


1 Department of Oral and Maxillofacial Surgery, NMCH, Nellore, Andhra Pradesh, India
2 Department of Neurosurgery, NMCH, Nellore, Andhra Pradesh, India

Date of Web Publication16-Sep-2013

Correspondence Address:
Mohammad Akheel
Block 5, 4H, VGN Laparasiene, Nolambur, Mogappair West, Chennai - 600 037
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-9727.118246

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  Abstract 

Elongation of the styloid process is a very rare clinical phenomenon, affecting 4% of the overall population. The etiopathogenesis of this syndrome is still an enigma although several theories are proposed in the literature. Clinical manifestations of this syndrome occur only in 4-10% of the affected population. If it occurs, an appropriate clinical diagnosis can be made. Most commonly, this syndrome is seen in routine radiographic investigations with elongation of the styloid process, which may be unilateral or bilateral. We report a case of a 45-year old female who had dysphagia with vague cervicofacial pain and was diagnosed with Eagle's syndrome.

Keywords: Carotid artery syndrome, eagle syndrome, elongated styloid process, serum calcium


How to cite this article:
Akheel M, Tomar S. Eagle syndrome. Muller J Med Sci Res 2013;4:124-6

How to cite this URL:
Akheel M, Tomar S. Eagle syndrome. Muller J Med Sci Res [serial online] 2013 [cited 2021 Dec 4];4:124-6. Available from: https://www.mjmsr.net/text.asp?2013/4/2/124/118246


  Introduction Top


The styloid process is a bilateral cylindrical slender projection attached to the base of the skull on the temporal bone that extends downwards, forwards and medially. The normal length of the styloid process is 25-30 mm. [1],[2],[3],[4] When it exceeds the normal length, it is aptly called as elongated styloid process. This was first described by an Italian surgeon Pietro Marchetti in 1652. In 1937, Watt W. Eagle coined the term styalgia, which means pain associated with an elongated styloid process and is hence named as Eagle's syndrome. [2] This styloid process gives attachment to many important anatomical structures. From the tip of the process arises the stylohyoid ligament and muscle, which attaches itself to the lesser cornua of the hyoid bone. The anterior surface of the styloid process gives rise to styloglossus muscle. Medial to the styloid process arises an important vascular structure named external carotid artery. Elongation of the styloid process causes pressure on the external carotid artery and is hence called as carotid artery syndrome. Elongation of the styloid process is seen in 4% of the population, which is believed to be caused due to ossification of the styloid process that has retained its embryonic remnants of cartilage. [5] We report a case of a 45-year-old female who had dysphagia with vague cervicofacial pain and was diagnosed with bilateral Eagle's syndrome.


  Case Report Top


A 45-year-old female patient reported to the Division of Oral and Maxillofacial Surgery complaining of difficulty in swallowing and vague discomfort in the throat from the past 7 months. She also had a complaint of intense pain on turning the head on either side. On clinical examination, there was a tender, bony hard mass palpable in the tonsillar fossa bilaterally. No cervical lymphadenopathy was present. She had no history of trauma or any previous surgeries. The remainder of her medical history was non-contributory. Radiographic investigations included an orthopantomogram, which revealed a radiopaque thick band-like structure measuring about a total length of 8 cm arising from the base of the skull till the lesser cornua of the hyoid bone that was identified as an elongated styloid process bilaterally, with 5 cm being styloid process and 3 cm being ossified stylohyoid ligament [Figure 1]. Her hematological investigations were within normal limits but with mild raise in the serum calcium levels (12 mg/dl). As the patient was not willing for a surgery, she was managed conservatively with non-steroidal anti-inflammatory medications and corticosteroids for 5 days.
Figure 1: Orthopantomogram showing the bilateral elongated styloid process

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  Discussion Top


The components of the styloid process are derived from the first and second branchial arches in four distinct segments, namely, tympanohyal, stylohyal, ceratohyal and hypohyal. These segments combine together and ossify into two parts. The styloid process develops from tympnaohyal and stylohyal segments that fuse at puberty of an individual. The hypohyal segments gives rise to the lesser cornua of the hyoid bone. The stylohyoid ligament connects these two segments arising from the ceratohyal segment. [6] The etiology of elongation of the styloid process is still an enigma. Several theories have been proposed, such as (a) congenital elongation of the styloid process due to persistence of an embryonic cartilage of the stylohyal (one of the embryologic precursors of the styloid) segment, (b) calcification of the stylohyoid ligament by an unknown process, (c) growth of osseous tissue at the insertion of the stylohyoid ligament [5] and (d) ectopic calcification due to abnormal metabolism of Ca, P and vitamin D. In the case reported here, the patients serum calcium levels were increased, which might be the reason of ossification of the stylohyoid ligament.

The pathophysiological mechanism of this syndrome is still unclear. There are some theories put forward by various authors in the literature. They are (a) traumatic fracture of facial skeleton or skull base involving the styloid process causing proliferation of granulation tissue, which causes pressure on the surrounding anatomical vital structures, [7] (b) compression of adjacent nerves, the glossopharyngeal, lower branch of the trigeminal or chorda tympani, (c) degenerative and inflammatory changes in the tendonious portion of the stylohyoid insertion, called insertion tendonitis or styloid tendonitis, (d) irritation of the pharyngeal mucosa by direct compression of the cranial nerves V, VII, IX and X and (e) impingement of the carotid vessels, mainly the external carotid artery producing irritation of the sympathetic nerves in the arterial sheath. [7]

Elongation of the styloid process is thought to present in 4% of the population, but only a small percentage (4-10.3%) are symptomatic and occur bilaterally in 75% of the affected population. Clinical manifestation of Eagle's syndrome is an aggregate of symptoms that commonly includes dysphagia, cervicofacial pain, foreign body sensation, pain in throat and ear ache. Other symptoms include vertigo, tinnitus, dysphonia, carotidynia, pain on turning the head, reduced mandibular opening and change in voice, hypersalivation and alteration in taste. [7],[8]

Clinical diagnosis is made by palpation of the styloid process in the tonsillar fossa, which is indicative of elongated styloid processes of which normal length are not normally palpable. Palpation of the tip of the styloid should exacerbate existing clinical symptoms. Confirmation is made by radiographic investigations. Most frequently, a panoramic radiograph is used to determine whether the styloid process is elongated. In case of unilateral elongation, it can be confirmed by taking a lateral cephalogram for a more clear image of the elongated styloid process. While reviewing these radiographs, Frommer et al. [9] indicated that the normal length of the styloid process is 25-30 mm whereas the length of the elongated styloid process is more than 30 mm. In our case, it was 8 cm in length, with a styloid process of 5 cm and a stylohyoid ligament of 3 cm bilaterally.

Treatment of Eagle's syndrome is both surgical and non-surgical. Non-surgical treatments include patient counseling of the clinical symptoms, non-steroidal anti-inflammatory medications and corticosteroids. [10] Surgical treatment is by two approaches. The transpharyngeal approach is approached intraorally. This approach was preferred by W. Eagle and the elongated portion of the styloid process was removed. [11] The advantage of this technique is to prevent external scarring but the disadvantages are poor visualization with deep space neck infections. [11] Another approach is the extraoral retromandibualr approach, which is considered to be superior for excellent visualization and accessibility of the surgical field.


  Conclusion Top


Identification and diagnosis of the elongated styloid process needs proper clinical and radiological examination. The exact cause of Eagle's syndrome is still a controversy. However, treatment of this syndrome is planned based upon the degree of severity of the clinical symptoms.

 
  References Top

1.Sokler K, Sandev S. New classification of the styloid process length - Clinical application on the biological base. Coll Anthropol 2001;25:627-32.  Back to cited text no. 1
    
2.Prasad KC, Kamath MP, Reddy KJ, Raju K, Agarwal S. Elongated styloid process (Eagle's syndrome): A clinical study. J Oral Maxillofac Surg 2002;60:171-5.  Back to cited text no. 2
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3.Monsour PA, Young WG. Variability of the styloid process and stylohyoid ligament in panoramic radiographs. Oral Surg Oral Med Oral Pathol 1986;61:522-6.  Back to cited text no. 3
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4.Ilgüy M, Ilgüy D, Güler N, Bayirli G. Incidence of the type and calcification patterns in patients with elongated styloid process. J Int Med Res 2005;33:96-102.  Back to cited text no. 4
    
5.Kim E, Hansen K, Frizzi J. Eagle syndrome: Case report and review of literature. Ear Nose Throat J 2008;87:631-3.  Back to cited text no. 5
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6.Rodriguez-Vazquez JF, Merida-Velasco JR, Verdugo-Lopez S, Sanchez-Montesinos I, Merida-Velasco JA. Morphogenesis of the second pharyngeal arch cartilage (Reichert's cartilage) in human embryos. J Anat 2006;208:179-89.  Back to cited text no. 6
    
7.Balasubramanian S. The ossification of the stylohyoid ligament and its relation to facial pain. Br Dent J 1964;116:108-11.  Back to cited text no. 7
    
8.Rechtweg JS, Wax MK. Eagle's syndrome: A review. Am J Otolaryngol 1998;19:316-21.  Back to cited text no. 8
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9.Frommer J. Anatomic variations in the stylohyoid chain and their possible clinical significance. Oral Surg Oral Med Oral Pathol 1974;38:659-67.  Back to cited text no. 9
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10.Baugh RF, Stocks RM. Eagle's syndrome: A reappraisal. Ear Nose Throat J 1993;72:341-4.  Back to cited text no. 10
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11.Chase DC, Zarmen A, Bigelow WC, McCoy JM. Eagle's syndrome: A comparison of intraoral versus extraoral surgical approaches. Oral Surg Oral Med Oral Pathol 1986;62:625-9.  Back to cited text no. 11
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