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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 28  |  Issue : 2  |  Page : 117-119

Eagle syndrome with multiple cranial nerve involvement


Department of Otorhinolaryngology, Regional Institute of Medical Sciences, Imphal, Manipur, India

Date of Web Publication18-Sep-2014

Correspondence Address:
Dr. Raj Kumar Bedajit
Department of Otorhinolaryngology (ENT), Regional Institute of Medical Sciences, Lamphelpat, Imphal - 795 004, Manipur
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-4958.141100

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  Abstract 

Eagle's syndrome (ES) represents a group of symptoms that includes recurrent throat pain, dysphagia, referred otalgia and neck pain possibly caused by elongation of the styloid process or ossification of the stylohyoid ligament. It is typically seen in patients after pharyngeal trauma or tonsillectomy. An elongated styloid process occurs in about 4% of the general population, while only a small percentage (between 4-10.3%) of these patients are symptomatic. So the true incidence is about 0.16%, with a female-to-male predominance of 3:1. The symptoms related to this condition can be confused with those attributed to a wide variety of facial neuralgias. We describe the case of a 45-year-old woman who experienced unremitting left neck pain following parotidectomy. This case is being reported because of its unusual presentation, difficulty in diagnosis, its rarity in occurrence and for academic interest.

Keywords: Styloid process, facial Neuralgias, Multiple cranial nerve palsies


How to cite this article:
Bedajit RK, Priyokumar O, Abhilash R, Kumar S. Eagle syndrome with multiple cranial nerve involvement. J Med Soc 2014;28:117-9

How to cite this URL:
Bedajit RK, Priyokumar O, Abhilash R, Kumar S. Eagle syndrome with multiple cranial nerve involvement. J Med Soc [serial online] 2014 [cited 2020 May 29];28:117-9. Available from: http://www.jmedsoc.org/text.asp?2014/28/2/117/141100


  Introduction Top


Eagle syndrome (ES) represents a constellation of symptoms, most notably facial pain, that are believed to be related to an elongated styloid process or calcified stylohyoid ligament. ES is a rare entity which is not commonly suspected in clinical practice. [1] The reported incidence of an elongated styloid process, defined as one that is longer than 25 mm, ranges from 1-7%of the population, yet only 4-7% of these patients experience related symptoms. [2] Patients with ES may present with sore throat, ear pain, or even with foreign body sensation in the pharynx secondary to pharyngeal and cervical nerve interactions. The syndrome is usually seen in patients after pharyngeal trauma or tonsillectomy. Since the symptoms are variable and non-specific, patients seek treatment in several different clinics such as otolaryngology, family practice, neurology, neurosurgery, dentistry and psychiatry. [3]

In the clinical examination of patients with facial pain and/pain in the cervical region, investigation of styloid process is rarely included. Consequently, styloid process pathology as a possible source of referred pain is often overlooked. Here we report an unusual case of eagle syndrome with multiple cranial nerve involvement that could cause difficulty in the differential diagnosis of facial pain.


  Case Report Top


A-45-year old female reported in our institute with complaints of left sided facial pain, earache, hoarseness, difficulty in swallowing and foreign body sensation in throat. She had undergone total parotidectomy for adenoid cystic carcinoma 1 year before. Symptoms developed 2 months after operation, when she developed severe lanciating hemifacial pain on the left side of face and neck. The pain was localized to the angle of lower jaw, radiating to left ear and anterolateral aspect of neck. Pain aggravated on swallowing, coughing, pressing the neck and occasionally on movement of neck from side to side. She developed hoarseness 4 months after the operation and difficulty in swallowing with nasal regurgitation while drinking water. On examination, there was severe pain on movement of head and on pressing the left tonsillar fossa. She had left vocal cord paralysis, left palatal paresis and deviation of tongue towards left, showing cranial nerve 9, 10 and 12 involvement. Patient also developed left sided facial paralysis before parotid surgery [Figure 1]. X-ray shows elongated left styloid process, measuring 3.9 cm [Figure 2].Videolaryngoscopy showed left vocal cord paralysis. Three dimensional computed tomography (3D CT) showed elongated styloid process with fibrosis around the left styloid process which confirmed the diagnosis [Figure 3]. Patient was treated conservatively with analgesic and anti-inflammatory medicines as she refused surgery.
Figure 1: Patient with left sided hypoglossal nerve paralysis


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Figure 2: Plain X ray showing left enlongated styloid process (39.25mm)


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Figure 3: 3D reconstruction CT showing elongated styloid process


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  Discussion Top


Eagle syndrome, sometimes called styloid or stylohyoid syndrome, is defined as the symptomatic elongation of the styloid process or mineralization (ossification or calcification) of the stylohyoid ligament complex. [4],[5] The syndrome was described as early as 1870, but it attained its eponymic designation after Eagle categorized the syndrome into two distinct types - the classic type and the carotidartery type - in 1937. [6] The classic type is seen after tonsillectomy, when the patient experiences pain that is attributable to the stimulation of cranial nerves V, VII, IX and X. In the carotid artery type, symptoms occur when the elongated styloid process stimulates the sympathetic nerves in the carotid sheath. [2],[6]

Over a twenty year period, Eagle reported over 200 cases and explained that the normal styloid process is approximately 2.5-3.0 cm inlength. He observed that slight medial deviation of the styloid process, could result in severe symptoms of atypical facial pain. [7] However, the mere presence of an elongated styloid processor mineralization of the stylohyoid complex radiographically in the presence of cervico-pharyngeal pain does not automaticallyconfirm a diagnosis of Eagle Syndrome. [8]

The cause of onset of pain in patients previously free ofsymptoms is unknown, but several mechanisms have been proposed that include rheumatic styloiditis caused by pharyngeal infections, trauma, tonsillectomy, and involutional changes associated with ageing (e.g., degenerative cervical discopathy, which may shorten the cervical spine and alter the direction of the styloid process). Also stretching and fibrosis involving the fifth, seventh,ninth and tenth cranial nerves in the post-tonsillectomyperiod is believed to be the major cause. [9]

At the clinical examination, the palpation of the styloid process in the tonsillar fossa indicates elongation because normalprocesses are not palpable. In ES this maneuver reproducesor exacerbates the pain. [10] As far as radiography is concerned, at present the oropharyngeal CT with 3D reconstruction allows images of optimaldefinition making the diagnosis easier than in the past. [11] An additional proof is a temporary relief of the symptomsfollowing the local infiltration of lidocaine. [12]

Numerous therapeutic approaches, both conservative and invasive, have been used to treat Eagle syndrome inthe setting of an elongated styloid process. Nonsurgical therapies have not consistently provided long-term relief, so surgery continues to be the mainstay of treatment. The earliest reported intervention involved manual transoral fracture, but the effect of this procedure on symptoms proved to be unpredictable. [13],[14] The main controversy today involves the surgical approach.

The transoral approach, introduced by Eagle himself, offers the advantages of a direct approach and no external scarring. [2] Its limitations include poor visibility leading to an increased risk of neurovascular injury, oral flora contamination, and the potential for postoperative airway obstruction secondary to pharyngeal edema. [3]

The transcervical approach offers better exposure and better visualization of the neurovascular structures in the surgical site, better field sterility, and the ability to excise a larger portion of the styloid process. [13] On the other hand, this approach does require an external incision, and the recovery period may be longer. The transcervical approach also places the marginal mandibular branch of the facial nerve at risk of transection, which may result in postoperative lip weakness, but this risk can be minimized by careful dissection in the appropriate plane. [3]


  Conclusion Top


The elongated styloid process syndrome can be diagnosed by adetailed history, physical examination, and radiological investigations.It can be confused or mistaken for many other conditionsthat must be excluded. Resection of the elongated styloid process is the treatment of choice. An awareness ofpain syndromes related to the styloid process is important to all health practitioners involved in the diagnosis and treatment of head and neck pain to rationalize the line of management.

 
  References Top

1.Fini G, Gasparini G, Filippini F, Becelli R, Marcotullio D. The long styloid process syndrome or Eagle's syndrome. J Craniomaxillofac Surg 2000;28;123-7.  Back to cited text no. 1
    
2.Eagle WW. Elongated styloid process; symptoms and treatment. AMA Arch Otolaryngol 1958;67:172-6.  Back to cited text no. 2
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3.Boscainos PJ, Papagelopoulos PJ, Goudelis G, Partsinevelos A, Nikolopoulos K, Korres DS. Eagle's syndrome. Orthopedics 2004;27:423-5.  Back to cited text no. 3
    
4.Monsour PA, Young, WG, Barnes PB. Styloid-stylohyoid syndrome: A clinical update. Aust Dent J 1985;30:341-5.  Back to cited text no. 4
    
5.Chouvel P, Rombaux P, Philips C, Hamoir M. Stylohyoid chain ossification: Choice of the surgical approach. Acta Otorhinolaryngol Belg 1996;50:57-61.  Back to cited text no. 5
    
6.Eagle WW. Elongated styloid process: Report of two cases. Arch Otolaryngol l937;25:584-7.  Back to cited text no. 6
    
7.Eagle WW. Symptomatic elongated styloid process; report of two cases of styloid process-carotid artery syndrome with operation. Arch Otolaryngol 1949;49:490-503.  Back to cited text no. 7
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8.Breault MR. Eagle's syndrome: Review of the literature and implications in craniomandibular disorders. Cranio 1986;4:323-37.  Back to cited text no. 8
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9.Ceylan A, Koybasioglu, Celenk F, Yilmaz O, Uslu S. Surgical treatment of elongated styloid process: Experience of 61 cases. Skull Base 2008;18:289-95.  Back to cited text no. 9
    
10.Kaufman SM. Elzay RP, Irish ER. Styloid process variation. Radioiogic and clinical study. Arch Otolaryngol 1970;91:460-3.  Back to cited text no. 10
    
11.Keur JJ, Campbell JP, McCarthy JK Ralph WJ. The clinical significance of the elongated styloid process. Oral Surg Oral Med Oral Pathol 1986;61:399-404.  Back to cited text no. 11
    
12.Lee S, Hillel A. Three-dimensional computed tomography imaging of Eagle's syndrome. Am J Otolaryngol 2004;25:109.  Back to cited text no. 12
    
13.Diamond LH, Cottrell DA, Hunter Ml, Papageorge M. Eagle's syndrome: A report of 4 patients treated using a modified extraoral approach. J Oral Maxillofac Surg 2001;59:1420-6.  Back to cited text no. 13
    
14.Glogoff M, Baum SM. Cheifertz I. Diagnosis and treatment of Eagle's syndrome. J Oral Surg 1981;39:941-4.  Back to cited text no. 14
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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