Eagle’s syndrome

Eagle’s syndrome, defined by Eagle in 1949, is characterised by morphological abnormality/ossification of the styloid process. The average length of the styloid process is 20-30 mm in the adult Caucasians and 15.4-18.8 mm in the Asian population. An elongated styloid process is defined by being at least 30 mm long. The clinical picture is composed of recurrent throat and neck pain, radiating into the ear and dysphagia. Symptoms can be bilateral or, more frequently, unilateral. The incidence is 4-8 per 10,000 people.

Eagle’s syndrome

The aetiology is not well defined and a number of theories have been suggested, such as congenital elongation due to the persistence of an embryonic cartilaginous outgrowth, calcification of the stylohyoid ligament and formation of bone tissue at the insertion of the ligament. This clinical picture might also be seen in patients after tonsillectomy.

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Eagle’s syndrome is related to the presence of an abnormally elongated styloid process with/without aberrant direction and/or ossificated styloid ligament. Cranio-facial pain resembles glossopharyngeal neuralgia and it is secondary to the irritation of the surrounding neurovascular and muscular anatomical structures (carotid artery, cranial nerves and muscles). It has been hypothesised that the pain deriving from the elongated styloid process may be due to compression of the glossopharyngeal nerve as it passes over the superior constrictor muscle. Rare cases of internal carotid artery compression/kinking have been described. The styloid process may also compress the sympathetic nerve fibres, causing Horner syndrome. The differential diagnosis should include glossopharyngeal and trigeminal neuralgia, temporal arteritis, migraine, cluster headache, myofascial pain dysfunction syndrome, pain related to un-erupted third molars, cervical arthritis, tumours and ill-fitting or missing dentures.

64-channel MDCT with multi-planar reconstructions (MPR) and 3D volumetric reconstructions (3D-VR). Left- (A) and right-sided view (B) of the temporomandibular joint (closed mouth). Left- (C) and right-sided view (D) of the temporomandibular joint (open mouth).

The condition is more common in women and in patients older than 50 years. Mixed, non-specific symptoms, the absence of a clear aetiological link and the scant knowledge about this clinical entity often delay diagnosis, as described by our experience. Physical examination and clinical history are useful diagnostic tools. An elongated styloid process may be palpated during intraoral examination and may provoke the pain. Correct indication to radiological investigation, dedicated technology and experience are needed in order to reach a diagnosis. Although plain skull radiographs might be sufficient to reveal the anatomical abnormality, CT of the head/neck and especially 3D-CT scan is considered as the gold standard for visualisation of the anatomically complex styloid process, as it avoids the problems of obscured overlapping anatomy. Moreover, it underlines the styloid process angulation, which is crucial for the surrounding anatomical relationships. Some controversy exists in the literature as to how many patients with an elongated styloid apophysis, at radiologic examination, do not exhibit any clinical symptoms. However, the surgical approach in patients with a diagnosis of Eagle’s syndrome is quite conclusive: surgical styloidectomy has, in fact, a cure rate of 80%.

Watt Weems Eagle (1898-1980), American otorhinolaryngologist.

Watt Weems Eagle

Watt Weems Eagle (1898-1980) was an American otolaryngologist who first described Eagle syndrome, a rare clinical entity that presents with a triad of dysphagia, cervical pain, and foreign-body sensation in the throat. The aim of this study is to present a historical vignette on Eagle's life and on the syndrome that became eponymous with his name.

Watt Weems Eagle was born in Statesville, North Carolina, in 1898. He obtained his medical degree and completed a residency in otolaryngology at Johns Hopkins. At Duke, Eagle served as the first chief of the ENT division from 1930-1949. In several publications published in the late 1930s, Eagle was the first to systematically describe the clinical syndrome and surgical treatment for elongated styloid processes, and this syndrome has since been associated with his name. The classical form presents with persistent unilateral pharyngeal pain worsened by swallowing. The vascular form, which is of interest to neurosurgeons, is characterized by compression of the internal carotid artery by an elongated styloid process.

Watt Eagle was an outstanding American surgeon and otolaryngologist who spent the majority of his career at Duke Medical Center. He is known for defining Eagle syndrome, a rare clinical entity with a constellation of neuropathic and vaso-occlusive symptomatology caused by abnormal elongation or angulation of the styloid process.

References

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