Eagle's syndrome

Síndrome de Eagle

Eagle's syndrome (ES) consists of craniofacial and neck pain due to elongation of the styloid process and/or calcification of the stylohyoid ligament¹. The normal length of the styloid process ranges from 2 to 3 cm and it is hardly palpable. ES is usually an asymptomatic disease that mostly affects women aged 30 to 50 years.

According to Eagle, two types of the syndrome have been described²: (A) the classic syndrome characterized by pain in the tonsillar fossa, sometimes associated with dysphagia and hypersalivation, and often followed by tonsillectomy and (B) the styloid-carotid syndrome: the styloid process compresses the carotid arteries and exerts particular pressure on its sympathetic fibers. This subtype is not correlated with tonsillectomy.

We report the case of a young patient with ES and a styloid process of normal lenght, but with significant ligament calcification.

CASE

A 20-year-old female patient presented at the Neurology Service with a continuous, daily, pressure-type pain on the left side of the neck radiating to frontal and temporal regions. There was neither history of trauma or previous surgery, nor identifiable trigger points. Physical examination revealed pain on tonsillar fossa at palpation, as well as on the left side of the neck. The neurological examination including the cranial nerves was normal. Three-dimensional computed tomography (CT) scan of the neck showed bilateral stylohyoid ligament calcification of 1.9 cm on the right side and 1.2 cm on the left side (Fig 1). The length of the styloid process added to the length of the calcification was, respectively, 4.1 and 3.7 cm (Fig 2). The symptom of pain was solved after administration of ibuprofen for two months.

Fig 1.
3D-CT scan image showing bilateral calcification of the stylohyoid ligament.: right stylohyoid ligament with 1.9 cm; left stylohyoid ligament with 1.2 cm.

Fig 2.
CT scan image showing increased styloid processes: right with 4.1 cm; left with 3.7 cm.

DISCUSSION

ES is a rare condition of unknown pathophysiology and etiology². It can be idiopathic, congenital (due to persistence of cartilage elements of the precursors of the styloid process) or acquired³.

Approximately 4% of the population has elongated styloid process, but only 4 to 10.3% of this group has its symptoms⁴.

Although many authors believe that this syndrome is more typical in adult life, the symptoms' onset of the patient herein described began in her 20s.

Treatment for ES includes non-steroidal anti-inflammatory drugs, steroids and applications of anesthetic in the tonsillar fossa⁴, in addition to surgical approach consisting of removal of the styloid process intra or extraorally.

The patient reported full improvement with non-steroidal anti-inflammatory drugs. However, it is not known whether the symptoms may return in old age.

Dissection, aneurysm and pseudoaneurysm of the carotid artery may occur in the course of ES. Further studies are needed to investigate the relationship between the elongated styloid process and the development of such complications⁵.

References

• ¹
  Savranlar A, Uzun L, Uğur MB, özer T. Three-dimensional CT of Eagle's syndrome. Diagn Interv Radiol 2005;11:206-209.
• ²
  Politi M, Toro C, Tenani G. A rare cause for cervical pain: Eagle's syndrome. Int J Dent 2009;2009:781297.
• ³
  Raina D, Gothi R, Rajan D. Eagle syndrome. Indian J Radiol Imaging 2009;19:107-108.
• ⁴
  Murtagh RD, Caracciolo JT, Fernandez G. CT findings associated with Eagle syndrome. AJNR Am J Neuroradiol 2001;22: 1401-1402.
• ⁵
  Dao A, Karnezis S, Lane JS, Fujitani RM, Saremi F. Eagle syndrome presenting with external carotid artery pseudoaneurysm. Emerg Radiol 2011;18:263-265.

Publication Dates

• Publication in this collection
  Apr 2013

History

• Received
  23 Apr 2012
• Received
  18 Oct 2012
• Accepted
  25 Oct 2012