|Year : 2017 | Volume
| Issue : 2 | Page : 66-70
Eagle's Syndrome: Our experiences in a tertiary care teaching hospital of Eastern India
Santosh Kumar Swain1, Asirbad Jena1, Mahesh Chandra Sahu2, Anwesha Banerjee3
1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
2 Department of Directorate of Medical Research, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
3 Department of Anesthesiology, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
|Date of Web Publication||22-Jan-2018|
Prof. Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
Source of Support: None, Conflict of Interest: None
Introduction: Eagle's syndrome (ES) is defined as the elongation of styloid process or the calcification of the stylohyoid ligament causing clinical manifestations such as throat pain, odynophagia, dysphagia, headache, and irradiation of pain into the ear and neck pain. The clinician misses the diagnosis due to nonspecific clinical presentations. Objective: To study detail clinical profile and management of the patients with ES in a tertiary care teaching hospital of eastern India. Materials and Methods: Twenty-one patients with ES were included in our study. Diagnosis of the ES was based on clinical presentations and three-dimensional computed tomography (3D CT scan). All were treated through transoral approach under general anesthesia. Results: 3D CT is very useful for preoperative estimation of the styloid process length. There were no postoperative complications encountered. Chief symptoms of the patients were regressed after surgery. Conclusion: Clinical presentations and three dimensional CT are important for exact diagnosis of ES. The transoral approach is a safe and definitive treatment of ES.
Keywords: Eagle′s syndrome, elongated styloid process, throat pain
|How to cite this article:|
Swain SK, Jena A, Sahu MC, Banerjee A. Eagle's Syndrome: Our experiences in a tertiary care teaching hospital of Eastern India. J Head Neck Physicians Surg 2017;5:66-70
|How to cite this URL:|
Swain SK, Jena A, Sahu MC, Banerjee A. Eagle's Syndrome: Our experiences in a tertiary care teaching hospital of Eastern India. J Head Neck Physicians Surg [serial online] 2017 [cited 2019 Sep 15];5:66-70. Available from: http://www.jhnps.org/text.asp?2017/5/2/66/223762
| Introduction|| |
Eagle syndrome (ES) is a rare clinical entity consisting of pain in throat, foreign body sensation in throat, ear pain, and neck pain. ES occurs due to abnormally elongated styloid process or due to mineralization of Reichert's cartilage in the stylohyoid ligament. ES is also defined as the symptomatic elongation of the styloid process or calcification of the stylohyoid ligament. This syndrome was first described by Watt W. Eagle an Otorhinolaryngologist in 1937. The diverse nature and vague quality of the pain in ES, patient may initially go the general physician, neurologist, oral surgeon or otorhinolaryngologist or dentistry even to psychiatrist. In ES, the clinical presentations are variable and nonspecific. It ranges from throat pain, ear pain, foreign body sensation, neck pain or headache and so often ES diagnosed in late. Often, the patient is misdiagnosed and treated with different antibiotic and painkillers for treatment by local physicians. The clusters of clinical symptoms are due to abnormally elongated styloid process or stylohyoid chain calcification. Clinician often misses the diagnosis due to nonspecific clinical presentations. ES is a rare clinical entity and constitutes 4% of the population having elongated styloid process and calcified stylohyoid ligament. The diagnosis is usually made by palpating the tonsillar fossa for elongated styloid process. Clinicians should consider the ES when both the clinical presentations and imaging evidence support the diagnosis. Here, we study detail clinical profile and management of the patients of ES in a tertiary care teaching hospital of eastern India.
| Materials and Methods|| |
A retrospective study of 21 cases of ES was done during 2009–2016 at the otorhinolaryngology department of our tertiary care teaching hospital. All patients came with complaints of foreign body sensation in the throat, ear pain, neck pain, and vague pain at the tonsillar fossa. Out of 21 patients, twelve were female and nine were male. Examination of the oropharynx showed normal tonsils without any evidence of infections. As throat pain did not coincide with symptoms of tonsillitis, palpation of the upper part of the tonsillar fossa, but using gloved fingers revealed a sharp prick sensation. After clinical diagnosis of the elongated styloid process, orthopantogram (OPG) [Figure 1] was done in all the cases. One milliliter of 2% xylocaine was injected at the upper pole of tonsil where sharp prick sensation felt showed relieve of throat pain. Computed tomography (CT) scan of the skull base confirmed the diagnosis [Figure 2]. The length of styloid process was measured from the CT scan of the skull base. The maximum length of the styloid process is approximately forty millimeters. If its length is more than 40 mm, it is considered as elongated styloid process. After confirmation of elongated styloid process, the patient underwent tonsillectomy by dissection methods under general anesthesia. After removal of the tonsils, a sharp elongated styloid process palpated in the tonsillar fossa [Figure 3]. The tip of the styloid process was identified and dissected using dissector. The styloid process was stripped of all the attachments. The elongated styloid process was dissected till its base and tip was nibbled off by bone nibbler [Figure 4]. This procedure was repeated in the other side.
| Results|| |
Out of the 21 patients with ES in our study, 12 were female and 9 were male. The male to female ratio was 0.75:1 with female preponderance. The age ranges from 31 years to 71 years in our study with maximum incidence in between 50 and 60 years. The mean age group was 57.14 years. Fourteen patients (66.66%) presented with bilateral symptoms whereas seven patients (33.33%) presented with unilateral symptoms. The common symptoms were vague pain in the throat (8 patients), foreign body sensation in throat (3 patients), neck pain (2 patients), pain during swallowing (1 patients), headache (2 patients), pain in the ear (2 patients), pain in the temporomandibular joint (1 patients), and others (2 patients). Most of the patients were complaining more than one clinical symptom. The mean duration of symptoms was 1.12 years. There was treated by local physician for vague symptoms by different antibiotics and pain killers before confirmation of the diagnosis. The lengths of styloid process were between 39 mm to 47 mm with a mean of 43.28 mm. There is a definite correlation between the length of the styloid process and clinical manifestations. There were no postoperative complications such as bleeding, deep neck space infections, or neurovascular injuries. There were complete remissions of symptoms among 6 patients, whereas partial remission was seen among two patients. Detail clinical presentations and outcomes are given in [Table 1] and [Table 2].
|Table 1: Distribution of age, sex, clinical feature and postoperative output|
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| Discussion|| |
ES is also called as styloid or stylohyoid syndrome. It is due to elongation of the styloid process or calcification of the stylohyoid ligament. The styloid process is considered as normal when its length is <2.5 cm and called elongated when its length is longer than 4 cm. Female comprises 85% of patients and it occurs among the age group between the second and third decades of life. The elongated styloid process irritates nerves and blood vessels, causing pain in the surrounding area. Elongated styloid process is seen in about 4% of the population and causing symptoms in only small subgroup. The etiopathogenesis of ES is controversial. A previous trauma like tonsillectomy or long-standing irritation of the stylomandibular ligament like tendonitis or periostitis can cause ossifying hyperplasia of the styloid process. Few author suggested osseous metaplasia of the Reichert's cartilage residues or persistence of mesenchymal tissue causing formation of bone tissue in adults or ossification of stylohyoid ligament due to endocrine lesions. In few cases, ES occurred after fracture of styloid process. Fracture of the styloid process usually occur from the trauma although characteristic symptoms after avulsion fracture caused by severe coughing, sudden laughing, and epileptic seizures. The mechanism for the pain in the ES are: (1) compression of the glossopharyngeal nerve, lower branch of the trigeminal nerve, or the chorda tympani nerve by the elongated styloid process; (2) fracture of the styloid process or ossified stylohyoid ligament followed by granulation tissue formation and causes compression on the surrounding structures and leads to pain; (3) elongated styloid process causing impingement on the carotid vessels and produces irritation of the sympathetic nerves surrounding the carotid sheath; (4) insertion tendinosis which is due to degenerative and inflammatory changes of the tendinosis part of the stylohyoid muscle; (5) direct compression of the pharyngeal mucosa by the styloid process; and (6) stretching and fibrosis affecting the cranial nerves like 5th, 7th, 9th and 10th during posttonsillectomy period. The styloid process arises from the bottom of the petrous part of the temporal bone and crosses parapharyngeal structures such as internal carotid artery, internal jugular vein, maxillary artery, glossopharyngeal nerve, vagus nerve and branches of the facial and trigeminal nerve medial to the styloid process. The proximity of the styloid process to the carotid canal and jugular foramen affects the pathophysiology of ES. ES is suspected when patient complains persistent throat pain which is exacerbated by head rotations, tongue movement, swallowing, or chewing food. The throat pain may be associated with excess salivation and foreign body sensation in the throat in the affected side. Palpation of the tonsillar fossa triggers the pain in the affected side which alerts the clinician for possibility of the diagnosis of ES. The elongated styloid process may compress the external or internal carotid arteries and the perivascular sympathetic nerve fibers leading to the pain in neck and face during head rotation. The clinical diagnosis of ES is done by taking good history and physical examination. The proper examination of the oral cavity and oropharynx with placing the index finger at the tonsillar fossa with gentle pressure will give a palpatory feeling of the elongated styloid process. During palpation, if cause referred pain to the ear, face, or head give the likely diagnosis of an elongated styloid process. Normal anatomical length of styloid process usually not give rise to any clinical manifestations and also not palpable. Local anesthetic infiltration at the tonsillar fossa relieving pain is a diagnostic feature for the ES. If a patient is presenting with long-standing pain in head and neck area, clinician should consider a diagnosis of ES as a possible cause. The clinician tries to rule out the elongated styloid process by different clinical examination and imaging. If we fail to diagnose the disease, it leads to delaying the treatment of disease and further degrading the patient's quality of life. We did not found any neurovascular complications during and after surgery, and symptomatic improvement was done in all patients. However, the different complications through transoral approach are neck infections, poor visibility of the operative field and risk of injury to neurovascular structures and leaving short residual styloid process. The differential diagnosis of the ES is craniomandibular dysfunction, trigeminal neuralgia, glossopharyngeal neuralgia, temporal arthritis, migraine, histamine headache, pain due to impacted third molar, oropharyngeal tumor, carotid artery syndrome, and Ernest syndrome. ES is also a differential diagnosis of temporomandibular joint diseases. As the pain of the ES may occur at the temporomandibular joint region, preauricular area occurs by mandibular movements, headache, tinnitus, earache, dizziness, hypoacusis, and hyperacusis. The possibility of ES is taken into consideration in the differential diagnosis of orofacial pain. The diagnosis of ES is confirmed by imaging like plain radiographs such as lateral skull film, poster anterior skull film, oblique mandible film, and Towne's panoramic views. CT scan is helpful to study the anatomical relationships of the styloid process and surrounding vital structures such as vessels and nerves. CT scan with three-dimensional reconstruction and OPG give accurate diagnosis. There are different surgical approaches for resection of styloid process. Different extraoral approaches have been described in literature; among them, the submandibular, retroauricular, preaural as well as intraoral approaches. The treatment of ES may be surgical or medical. The medical treatment includes infiltration of local anesthetics or steroids or giving oral carbamazepine. However, the output of medical treatment is not satisfactory. The surgical approach for ES is partial removal of styloid process by intraoral or extraoral approach. The transoral approach is quicker and easier to perform and avoid extensive dissection as in cervical approach without any external scar. The transoral approach for styloid process excision is often criticized due to the chance of deep neck space infection and poor visibility of the operative field. The main advantages of this approach are absence of the external scar and avoidance of general anesthesia if patient had already done tonsillectomy surgery. The extraoral or transcervical approach is thought to be superior one by many surgeons due to better visualization of the operative field, so if any vascular lesion resolve without any problems  and less chance of the infections at the deep neck space. However, it may take longer operating time and external scar. We had done intraoral approach in all our cases without any complications except one presented with secondary hemorrhage which was controlled under general anesthesia.
| Conclusion|| |
ES is considered in the differential diagnosis of patients complaining oropharyngeal pain or foreign body sensation in the throat. The diagnosis of ES is confirmed by palpating the tonsillar fossa and doing regional imaging. The ES can be diagnosed by detailed history taking, physical examination, and radiological test. It is often mistaken for other conditions that must be excluded before treatment. Resection of the styloid process is the treatment of choice. Clinician and general practitioners should get awareness with pain syndrome related to ES which includes throat pain, neck pain, and head pain. All these nonspecific pain often give rise to cancer phobia which causes great emotional distress among the patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]