Eagle syndrome

Eagle syndrome

Anteroposterior and lateral radiographs of cervical spine showing ossification of the stylohyoid ligament on both sides
Classification and external resources
DiseasesDB 33542
eMedicine article/1447247

Eagle syndrome (also termed stylohyoid syndrome[1] styloid syndrome,[2] styloid-stylohyoid syndrome,[2] or styloid–carotid artery syndrome)[3] is a rare condition caused by an elongated or deviated styloid process and/or calcification of the stylohyoid ligament, which interferes with adjacent anatomical structures giving rise to pain.

Signs and symptoms

Possible symptoms include:

Usually the condition is present on only one side, however rarely it may be present on both sides.[4]

Two forms of eagle syndrome exists: The classic form and the vascular one. Patients with the classic "Eagle Syndrome" can present with unilateral sore throat, dysphagia, tinnitus, unilateral facial and neck pain, and otalgia. In patients with the vascular form of "Eagle syndrome", the elongated styloid process is in contact with the extracranial internal carotid artery. This can cause a compression (while turning the head) or a dissection of the carotid artery causing a transient ischemic event or a stroke.

Cause

The cause of the condition is unknown.

Diagnosis

Diagnosis is suspected when a patient presents with the symptoms of the classic form of "eagle syndrome" e.g. unilateral neck pain, sore throat or tinnitus. Sometimes the tip of the styloid process is palpable in the back of the throat. The diagnosis of the vascular type is more difficult and requires an expert opinion. One should have a high level of suspicion when neurological symptoms occur upon head rotation. Symptoms tend to be worsened on bimanual palpation of the styloid through the tonsillar bed. They may be relieved by infiltration of lidocaine into the tonsillar bed. Because of the proximity of several large vascular structures in this area this procedure should not be considered to be risk free.

Imaging is important and is diagnostic. Visualizing the styloid process on a CT scan with 3D reconstruction is the suggested imaging technique.[6] The enlarged styloid may be visible on an orthopantogram or a lateral soft tissue X ray of the neck.

It is worth noting that the styloid may be enlarged (>30 millimeters in length) in 4% of the population and only a small minority (~4%) of people with enlarged styloids have symptoms.

Treatment

In both the classic and vascular form, the treatment is surgical.[7] A partial styloidectomy is the preferred approach. Repair of a damaged carotid artery is essential in order prevent further neurological complications.

Regrowth of the stylohyoid process and relapse are a common occurrence.[5]

Epidemiology

Approximately 4% of the general population have an elongated styloid process, and of these about 4% give rise to the symptoms of Eagle syndrome.[8] Therefore, the incidence of stylohyoid syndrome may be about 0.16%.[8]

Patients with this syndrome tend to be between 30 and 50 years of age but it has been recorded in teenagers and in patients > 75 years old. It is more common in women, with a male:female ratio ~ 1:2.

History

The condition was first described by American otorhinolaryngologist Watt Weems Eagle in 1937.[8]

References

  1. ↑ Waldman SD (6 June 2013). Atlas of Uncommon Pain Syndromes. Elsevier Health Sciences. pp. 35–36. ISBN 1-4557-0999-9.
  2. 1 2 Bumann A; Lotzmann U (2002). TMJ Disorders and Orofacial Pain: The Role of Dentistry in a Multidisciplinary Diagnostic Approach. Thieme. p. 279. ISBN 978-1-58890-111-8.
  3. ↑ Hoffmann, E.; Räder, C.; Fuhrmann, H.; Maurer, P. (2013). "Styloid–carotid artery syndrome treated surgically with Piezosurgery: A case report and literature review". Journal of Cranio-Maxillofacial Surgery 41 (2): 162–166. doi:10.1016/j.jcms.2012.07.004. PMID 22902881.
  4. 1 2 3 4 5 Kamal, A; Nazir, R; Usman, M; Salam, BU; Sana, F (November 2014). "Eagle syndrome; radiological evaluation and management.". JPMA. The Journal of the Pakistan Medical Association 64 (11): 1315–7. PMID 25831655.
  5. 1 2 3 4 5 Scully C (21 July 2014). Scully's Medical Problems in Dentistry. Elsevier Health Sciences UK. ISBN 978-0-7020-5963-6.
  6. ↑ Karam C, Koussa S (December 2007). "[Eagle syndrome: the role of CT scan with 3D reconstructions]". J Neuroradiol (in French) 34 (5): 344–5. doi:10.1016/j.neurad.2007.08.001. PMID 17997158.
  7. ↑ Orhan KS, Güldiken Y, Ural HI, Cakmak A (April 2005). "[Elongated styloid process (Eagle's syndrome): literature review and a case report]". Agri (in Turkish) 17 (2): 23–5. PMID 15977090.
  8. 1 2 3 Petrović, B; Radak, D; Kostić, V; Covicković-Sternić, N (2008). "[Styloid syndrome: a review of literature].". Srpski arhiv za celokupno lekarstvo 136 (11-12): 667–74. PMID 19177834.
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