CASE 11059 Published on 26.07.2013

Bilateral Eagle syndrome

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

Cekaj E, Baruti E, Cekaj (Beno) I

1- Regional Hospital of Durres, Durres ALBANIA
2- Private dental Clinic
3- Private dental Clinic

Patient

48 years, male

Categories

Area of Interest Head and neck ; Imaging Technique CT

Procedure Diagnostic procedure ; Special Focus Congenital ;

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Clinical History

The patient presented to the dentist with recurrent pain in the oropharynx and face especially during chewing more on the right side. After checking his teeth an orthopantomogram (OPT) was requested. Based on the findings the patient went on to undergo computed tomography (CT) of the cervical spine.

Imaging Findings

On the OPT an elongated styloid process was noted on the left and calcification along the stylohyoid ligament on the right (on the right we thought it was the calcified ligament because its image was composed by two separated parts) (red arrows Fig. 1). This was confirmed on CT. The combination of calcified ligament and styloid process measured 62.6 mm on the right and the styloid on the left 57.5 mm (Fig. 2, 3)

Discussion

The Eagle syndrome refers to symptomatic elongation of the styloid process or calcified stylohyoid ligament. It is often bilateral. In most cases the cause is not known, however, the condition is sometimes associated with disorders causing heterotopic calcification like myositis ossificans progressiva (fibrodysplasia ossificans progressiva) or postraumatic ossification. It was first described by Watt Eagle in 1937. [1-4]
Eagle defined the length of a normal styloid process at 2.5-3.0 cm and >3cm is generally considered to be in keeping with Eagle syndrome, but different studies have shown a range of normality from 1.52 to 4.77 cm [5-12]
An elongated styloid process occurs in about 4% of the general population, while only a small percentage (between 4-10.3%) of these patients is symptomatic. [13] So the true incidence is about 0.16%, with a female-to-male predominance of 3:1. Bilateral involvement is quite common but does not always involve bilateral symptoms. No significant difference is detectable between the right and left sides.
The syndrome can be divided into two main sub types: due to compression of cranial nerves and due to compression of the carotid artery. Patients can have facial pain while turning the head, dysphagia, foreign body sensation, pain on extending tongue, change in voice sensation, hypersalivation, tinnitus or otalgia, visual symptoms, syncope, sympathetic plexus irritation (carotidynia), eye pain and parietal pain [1, 3, 13, 14, 15]
Imaging modalities are orthopantomography and 3D computed tomography. An advantage of orthopantomography (panoramic view) is that the entire length of the process can be seen very distinctly and its deviation can also be made out clearly. Conventional radiographs provide a rough idea of the anatomy, the actual diagnosis is difficult due to the superimposed anatomical structures. CT scanning (and in particular 3-dimensional CT reformation) represents an extremely valuable imaging tool in patients with Eagle syndrome, offering an accurate evaluation of the styloid process in relation to its anatomical relationship with the other head and neck structures, to its length and to its usefulness in surgical planning [1, 2, 3, 6, 8, 11]
Treatment can be surgical or non-surgical. Nonsurgical means medical therapy with analgesics, anticonvulsants, antidepressant or local infiltration with steroids or long-acting local anaesthetic agents. Surgical treatment may be with intraoral or extraoral approach. Our patient underwent surgery with intraoral approach with two surgeries within three weeks and now he is doing well. [16, 17]

Differential Diagnosis List

Eagle syndrome

Trigeminal neuralgia

Otitis

Temporomandibular disorders

Glossopharyngeal neuralgia

Salivary gland disease

Final Diagnosis

Eagle syndrome

References

[1] Murtagh RD, Caracciolo JT, Fernandez G (2001) CT findings associated with Eagle syndrome. Am J Neuroradiol 22 (7): 1401-2 (PMID: 11498437)

[2] Lorman JG, Biggs JR (1983) The Eagle syndrome. Am J Roentgenol 140 (5): 881-2 (PMID: 23610891)

[3] Lugmayr H, Krennmair G, Lenglinger F (1997) Ossifcation of the stylohyoid chain in computerized tomography—Eagle syndrome. Aktuelle Radiol 7:331–332 (PMID: 9467028)

[4] Eagle WW (1937) Elongated styloid processes: report of two cases. Arch Otolaryngol 47:584–87 (PMID: 12645126)

[5] Moffat DA, Ramsden RT, Shaw HJ (1977) The styloid process syndrome: aetiological factors and surgical management. J Laryngol Otol 91(4):279-294 (PMID: 856922)

[6] Kaufman SM, Elzay RP, Irish EF (1970) Styloid process variation. Radiologic and clinical study. Arch Otolaryngol 91(5):460-3 (PMID: 5442737)

[7] Lindeman P (1985) The elongated styloid process as a cause of throat discomfort. Four case reports. J Laryngol Otol 99(5):505-8 (PMID: 3998636)

[8] Correl R, Jensen J, Taylor J et al (1979) Mineralization of the stylohyoid-stylomandibular ligament complex: A radiographic incidence study. Oral Surg Oral Med Oral Pathol 48:286-291 (PMID: 116179)

[9] Langlais RP, Miles DA, Van Dis ML (1986) Elongated and mineralized stylohyoid ligament complex: a proposed classification and report of a case of Eagle\'s syndrome. Oral Surg Oral Med Oral Pathol 61(5):527-32 (PMID: 3459129)

[10] Montalbetti L, Ferrandi D, Pergami P, et al (1995) Elongated styloid process and Eagle\'s syndrome. Cephalalgia 15(2):80-93 (PMID: 7641256)

[11] Monsour PA, Young WG (1986) Variability of the styloid process and stylohyoid ligament in panoramic radiographs. Oral Surg Oral Med Oral Pathol 61(5):522-6 (PMID: 3086788)

[12] Balcioglu HA, Kilic C, Akyol M, Ozan H, Kokten G (2009) Length of the styloidprocess and anatomical implications for Eagle\'s syndrome. Folia Morphol (Warsz) 68:265-70 (PMID: 19950078)

[13] Eagle W (1948) Elongated styloid process: Further observation and a new syndrome. Arch Otolaryngol 47:630-640 (PMID: 18894764)

[14] Prasad KC, Kamath MP, Reddy KJ, Raju K, Agar-wal S (2002) Elongated styloid process (Eagle’s Syndrome) A clinical study. J Oral Maxillofac Surg 60:171–5 (PMID: 11815916)

[15] Rechtweg JS, Wax MK (1998) Eagle’s Syndrome: A Review. Am J Otolaryngol 19:316–21 (PMID: 9758180)

[16] Fini G, Gasparini G, Filippini F, Becelli R, Marco-tullio D (2000) The long styloid process syndrome or Eagle’s syndrome. J Craniomaxillofac Surg 28:123–7 (PMID: 10958426)

[17] Ceylan A, Koybasioglu A, Celenk F, Yilmaz O, Uslu S (2008) Surgical treatment of elongated styloid process: Experience of 61 cases. Skull Base 18:289–95 (PMID: 19240828)

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