Eagle Syndrome - Causes, Symptoms, Diagnosis, and Management

Introduction:

Eagle syndrome is a rare condition that causes pain in the face or the neck. This condition usually occurs due to problems related to the styloid or the stylohyoid process. The first case was described by an otolaryngologist, Watt W. Eagle. The symptoms were typically reported after a pharyngeal trauma or post-tonsillectomy. In the later years, the term “ styloid syndrome” was used to describe cervico-pharyngeal pain associated with a styloid process when there is no previous history of trauma. A study found that around four percent of people have an unusually long styloid process, and only 1 in 62,000 have symptoms. Eagle syndrome is more commonly seen in women than men, and around three times more women have symptoms than men.

What Is Eagle Syndrome?

Eagle syndrome is a condition caused by a calcified stylohyoid ligament or an elongated styloid process causing recurrent pain in the middle part of the throat (oropharynx) and the face. The styloid process is a cylindrical needle-like projection of different lengths. They are located anteriorly to the stylomastoid foramen on the right and left sides of the skull. A classic Eagle syndrome occurs after a trauma to the throat or post-tonsillectomy. It is otherwise known as elongated styloid process syndrome or styloid-stylohyoid syndrome.

What Are the Causes of Eagle Syndrome?

The exact cause of eagle syndrome is still debatable. In most cases, it is known to be caused due to an elongated styloid process. People may develop this long styloid process following a throat injury or surgery. In a few, it is not more than an anatomical difference or age-related changes. Basically, the long and elongated styloid process puts pressure on the throat and compresses the nearby nerves and blood vessels, causing pain. The other causes include:

• Tonsillectomy: After the tonsillectomy procedure, scars develop in and around the throat, putting pressure on the surrounding nerves and causing pain and a ringing sensation in the ears.

• Calcification of the Stylohyoid Ligament: There are cases when the calcium deposits on the stylohyoid ligament, which attaches to the styloid process. In this case, people usually do not experience symptoms, but some may experience pain or other unusual sensations.

What Are the Symptoms of Eagle Syndrome?

The classic Eagle syndrome is seen after a pharyngeal trauma or tonsillectomy characterized by dull and persistent pharyngeal pain that can be referred to the ear and aggravated by turning or rotating the head.

The second form of Eagle syndrome is the stylo-carotid syndrome, which is caused by the compression of the internal or external carotid artery. It is related to the pain along the regions where the artery is distributed, which is further aggravated by rotating or compressing the neck. There are cases when the patient shows no symptoms. But when symptoms appear, they include the following:

• The pain is usually on one side of the neck and the face.

• A shooting kind of pain from the throat to the ear.

• Difficulty in swallowing.

• A feeling that something is stuck in the throat.

• Pain while swallowing.

• Pain while turning the head towards one side.

• Throbbing pain in the jaw.

• Headache.

• Persistent buzzing or ringing in the ears.

• Dizziness.

• There are also other symptoms some people experience, like unusual sensations in the head or neck.

What Is the Pathogenesis of Eagle Syndrome?

The cause is often not well understood, but it has been suggested that the cause has the following underlying causes.

1. Developmental Factors:

• Congenital Elongation: Ectopiccranial is a condition in which some people are born with an elongated styloid process.
• Genetic Predisposition: It may be related to genetics, as some families may be more susceptible to the condition.

2. Post-Traumatic Factors:

• Trauma: Injury of the styloid process or structures around it could cause reactive osteogenesis and styloid process elongation.
• Surgical Trauma: Earlier operations in the neck or throat area for other diseases can lead to such consequences as elongation or calcification due to the formation of scar tissue and healing processes.

3. Inflammatory Factors:

• Chronic Inflammation: The chronic inflammation in the region may by time result in ossification and elongation of the styloid process or ligament.

How to Diagnose Eagle Syndrome?

Diagnosis of this condition requires the use of both physical examination and imaging tests.

1. Clinical Evaluation:

• Medical History: The seven areas of patient history that should be elaborated include throat discomfort or pain, the feeling that there is something stuck in the throat, pain while swallowing, facial discomfort, and earache.
• Physical Examination: Pressure applied over the tonsillar fossa may cause tenderness, and in some instances, the stretched styloid process may be felt.

2. Imaging Studies:

• X-ray: Radiographic examination, such as the lateral view of the skull, may show elongation of the styloid process.
• CT Scan: Allows for a clear appreciation of the styloid process and the stylohyoid ligament to determine the level of its elongation and calcification.
• 3D Reconstruction: CT with 3D reconstruction can provide accurate measurement and clarify the spatial relationship of the related structures.
• MRI: If used in conjunction with other imaging modalities, it can help to evaluate soft tissue inflammation and to exclude other diseases.

Are There Any Complications With Eagle Syndrome?

While Eagle syndrome itself can cause significant discomfort and pain, there are potential complications associated with the condition, especially if left untreated:

• Vascular Complications: Carotid artery stenosis causes giddiness, transient ischemic attacks – TIAs, or stroke.

• Neurological Complications: Peripheral phenomena such as compression of cranial nerves like the glossopharyngeal or vagus nerves can cause neuropathic pain and dysfunction.

• Swallowing Difficulties: Possible complications include dysphagia if the lesion is caused by mechanical obstruction or is neural.

• Voice Changes: Precisely, if the nerves that supply the vocal cords are affected, changes in the voice or the occurrence of hoarseness can be expected.

• Referred Pain: It is also known to manifest in the ear, face, and neck areas and thus presents a possibility of people being diagnosed with other diseases when they are suffering from migraines.

What Is the Workup Done for Eagle Syndrome?

• Complete Blood Count (CBC): Firstly blood work is recommended to exclude other possible diseases and conditions. A complete blood count (CBC) is recommended if an underlying infection is suspected.

• Orthopantomogram (OPG): In this diagnostic report, the entire length of the styloid process will be seen distinctly, and even the deviation can be made out clearly.

• Computed tomography (CT) Scan: Computed tomography is one of the best and most effective diagnostic methods used in patients with Eagle syndrome. It offers an accurate and clear, distinct evaluation of the styloid process in relation to the other head and neck structures.

How Is Eagle Syndrome Managed?

Surgical Management: The first and foremost recommended treatment is styloidectomy. This procedure is performed through the intraoral (through the mouth) approach or the extraoral approach (through the neck) to shorten the styloid process. During this intraoral procedure, the tonsils may be removed to access the styloid process. Usually, this procedure is considered safe and simple, and a scar can be avoided. In this extraoral approach, styloidectomy can also be carried through an opening through the neck, which usually leaves a scar. This procedure is carried through the connective tissues, so there is an increased risk of trauma to the surrounding structures. In recent years, an endoscope-assisted approach has been used. This is a minimally invasive procedure carried out for head and neck surgeries. In this procedure, a long, thin tube fitted with a camera is inserted to provide direct surgical access and satisfactory exposure.

Medical Management: The medical management of Eagle syndrome includes analgesics and anti-inflammatory medications, antidepressants, or steroids.

How Is the Outlook of Eagle Syndrome?

In a survey, it was realized that around 80 percent of people who have received treatment for this syndrome had been relieved irrespective of the type of treatment they have received. In the case of surgical management, the prognosis is even better, and for patients who choose not to go for surgery, their symptoms have improved with medical management, but Eagle syndrome may become a chronic condition in those cases. So, living with chronic pain can cause depression or anxiety. In such cases, they can get help from support groups or other forms of psychological support.

Conclusion:

Eagle syndrome is not a progressive condition, so will not cause other medical illnesses. However, Eagle syndrome is a treatable condition with a good prognosis for most people who take treatment. A multidisciplinary approach and early radiological diagnosis are advisable in these cases, and the diagnostic workup can be critical with the misleading symptoms. However appropriate diagnosis and treatment help improve the patient's quality of life.