Chronically misdiagnosed Eagle’s syndrome, treated through transoral styloidectomy: a rare case report

Introduction:

The stylohyoid ligament begins when the styloid process extends from the petrous temporal bone in a thin bony protrusion. Eagle’s syndrome (ES) is a condition related to either calcification of the calcifying the stylohyoid ligament or elongating styloid process The incidence of symptomatic ES is 0.16%, and it is more common in women. The reported study diagnosed ES and treated it surgically through transoral approach styloidectomy.

Case presentation:

A 39-year-old man who is a farmer and a driver was presented with complaints of ongoing, excruciating discomfort in the back of his left ear. Before the exam, he took a variety of drugs, various drugs 2 years without receiving a definitive diagnosis. Axial, coronal, and sagittal computed tomography scans of both petrous bones were analyzed, and the results showed aberrant styloid process elongation and calcification of the stylohyoid ligament

Discussion:

ES, shares many symptoms with other regional illnesses. Physicians frequently misdiagnose cases of ES and treat them without providing a conclusive diagnosis or treatment.

Conclusion:

ES diagnosis can be difficult for otolaryngologists and primary care providers due to its similarity with other regional illnesses. However, surgical intervention can result in consistent and significant symptomatic improvement when correctly diagnosed. The case presented in the report was successfully diagnosed as ES and treated surgically through transoral approach styloidectomy.

Introduction

Highlights

• Eagle’s syndrome (ES) is often misdiagnosed due to similarity with other illnesses.

• Imaging techniques like computed tomography scans aid in accurate diagnosis.

• Transoral approach styloidectomy results in significant improvement.

• Primary care providers may also face difficulty in ES diagnosis.

• Correct diagnosis of ES essential for successful surgical intervention.

The styloid process (SP) is a slender bony projection from the petrous temporal bone, from its end the stylohyoid ligament originates, it also provides attachment to three muscles and one other ligament. The internal jugular vein, internal carotid artery, and IX, X, XI, and XII cranial nerves surround this slender bony projection. Its morphological features and length vary among individuals ranging from 20 to 30 mm^1,2.

In 4% of the population, the SP elongates or the stylohyoid ligament calcifies, this condition was named “Eagle Syndrome” after Watt W Eagle, who described its first cases. Eagle’s syndrome (ES) could be due to trauma (i.e. trauma to soft tissues while tonsillectomy or rarely trauma from dental extractions), endocrinological dysfunction in post-menopausal women, in rheumatoid diseased patients as a degenerative process, and genetically transmitted anatomical variation, or unknown aetiology, or could be due to physiological process of aging^3–6.

Of all ES cases, the true incidence of symptomatic ES is 0.16%, with a female‑to‑male predominance of 3:1⁷. ES is commonly bilateral however symptoms are typically unilateral. Due to compression and or irritation of structures surrounding the elongated SP symptoms occur, which include neck and throat pain (pharyngodymia), a sensation of a foreign body in the pharynx, difficulty in swallowing (dysphagia), pain in swallowing (odynophagia), otalgia, headache, neurological symptoms (such as speech disturbance and or hemiparesis), or combination of these symptoms^1,2,8. Extending pain to the posterior teeth and abnormal findings, lead to misdiagnosis and so interoperate treatment such as exodontia was performed while the patient has ES⁹. The study reported a misdiagnosis of ES and treated it surgically through transoral styloidectomy. This report has been written under the SCARE criteria guidelines for case reports¹⁰.

Patient information

A 39-year-old male was admitted to the Hospital, with complaints of continuous severe pain in the back of the left retroauricular region and migration which was exacerbated. He had a middle-class income. He was educated from secondary school and married, he works as a farmer and also a driver. No previous surgeries were recorded

For 2 years before the examination, he used many medications for instance analgesics and antibiotics in an interrupted style without any precise diagnosis or any outcome. The patient did not have any sensitivity to medications used previously. No family history of a similar case was noted, and he did not have bad habits.

Clinical finding

On physical examination, the vital signs were stable. The temperature was 36.7°C, and pulse rate and blood pressure were 89 bpm, and 120\80 mmHg, respectively. SPO2 was 94% and Limited painful neck movement, on examining his eyes, he had blurry vision. Pharyngeal foreign body sensation, limited pain in the molar region of the mandibular movement, eating, and drinking were painful, tenderness (pain on palpation) was noted at the left retroauricular region, headache, dysphagia, chronic pain in the pharynx and voice changes

Diagnostic assessment

For optimum diagnosis, Non-Contrast computerized tomography (CT) scan both petrous bone (axial, coronal, sagittal) was assessed, which revealed abnormal elongation of SP/calcification of stylohyoid ligament, the right side measuring about 35 mm and the left side measuring about 41 mm with the encouragement of surrounding structures (Fig. 1). Also, Cone beam CT (CBCT) confirm it.

Figure 1.

Showing bilateral elongation of SP/calcification of stylohyoid ligament. SP, styloid process.

Therapeutic intervention

After thorough consideration of patient history, clinical and diagnostic findings, the case was treated. an experienced team of oral-maxillofacial surgeons and ear nose and throat specialists decided to do transoral styloidectomy of the left elongated SP (Fig. 2). The patient accepted the treatment 6 months after the diagnostic time, due to continuous non-relieving pain. Under general anaesthesia (GA), the procedure started with a bilateral tonsillectomy. Then, the left side (16 mm) of the elongated SP was resected (Fig. 3) and the whole area was sutured with vicryl 3\0 absorbable suture. In addition, the two bony exostoses on the soft palate (Torus Palatinus) were removed after exposing the area to a 7 mm-long double-Y-shaped flap soft palate (7 mm).

Figure 2.

Exposing the elongated SP via transoral approach. SP, styloid process.

Figure 3.

Left side (16 mm) of the.elongated styloid process.

Follow-up and outcome

During the postoperative stage, the patient received a Ceftriaxone vial (1×2 ) for 4 days. Paracetamol ampule 600 mg (1×1) for 4 days. MEBO ointment was used for the side of suturing for the acceleration healing process. After the surgery, the patient had several follow-ups, which relieved all the previous symptoms.

Discussion

ES, an uncommon clinical disease caused by SP elongation, has many symptoms with other local disorders. The clinical signs include posterior tooth discomfort and aberrant results⁹. So ES, which is rarely considered, should be included in the diagnosis and treating oropharyngeal and maxillofacial cases¹¹. The present case visited physicians several times for treatment but was misdiagnosed, and using medications for 2 years long led to a delayed diagnosis; however, he has neither a history of systemic disease nor any surgery or trauma.

A history, clinical symptoms, tonsillar fossa palpation, and pain reduction after local anaesthetic in the tonsillar region are typically used to make the diagnosis of ES. Additionally, it has been suggested that CT and CBCT are the best imaging techniques for determining the location, size, shape, and angulation of the SPs as well as their closeness to nearby neurovascular structures^12–15.

Methods of ES management include both surgical and conservative ones⁶. The last one comprises a prescription of various medications to reduce the pain and anxiety of the patient. Other ways are physical therapy to relax the constricted muscles due to the elongated SP. In most cases, the pain was not relieved or otherwise recurred within 6 months to a year¹⁴. In the present case, a definitive diagnosis was confirmed by the confluence of the patient’s history, and radiographic assessment. The surgical intervention of shortening of the elongated SP should be performed via an intraoral or transcervical approach^14,16–18. The patient accepted the surgical treatment via intraoral, 6 months after the diagnostic time, due to continuous non-relieving pain.

The transcervical method gives sufficient anatomic exposure, greater vision of neurovascular systems in the surgical site, improved field sterility, and the capacity to remove a bigger section of the SP, but it leaves an external scar. An external incision is needed, and healing may take longer. The additional disadvantages are GA and extensive fascial dissection with unpleasant paresthesias, which may cause postoperative lip weakness in the marginal mandibular branch of the facial nerve due to nerve compression during surgery^19,20.

The transoral styloidectomy advantages technique simplicity, less operation time, and no external scar formation, and sometimes it can be performed under local anaesthesia, but usually, it is performed under GA. The drawbacks of the approach include poor visualization of the field, especially in patients with limitation of mouth opening, lack of access particularly in the presence of tonsillar haemorrhage or deep neck infections, and postoperative risk of neurovascular lesions, infection, oedema causing swelling, and speech alterations^{5,14,16,21,22}. The choice of the approach is often based on the surgeon’s experience. For the present case, the maxillofacial specialist decided to intraoral styloidectomy of the left elongated SP to avoid the risk of facial nerve or branch injury. While the patient was prepared for an external transcervical approach if we were not successful transorally.

Conclusion

ES diagnosis can be difficult not only for primary care providers but also for otolaryngologists. However, when correctly diagnosed, surgical intervention can result in consistent and significant symptomatic improvement. Physicians should include ES when diagnosing neck and throat complaints to prevent misdiagnosis. CT and CBCT radiographs should also confirm the diagnosis. Surgeon expertise and patient condition should determine surgical method. Finally, long-term follow-up ensures therapy efficacy and detects problems.

Ethical approval

The authors confirm that this study was prepared by COPE rules and regulations. The manuscript was approved by the ethical committee of Baxshin Research Center. (BRC0201023) dated 1 Feb 2023.

Consent

Informed consent was obtained from the patient before the preparation of the case report for publication (including clinical photographs), and the author/s endeavoured to ensure anonymity.

Source of funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Authors’ contributions

O.S.H. and A.B.R.: concept, study design, and patient follow-up. M.J.R., M.K.R., L.L.A., R.A.G., N.Q., and P.A.H.: literature review and write a draft manuscript. J.M.A.: discussion, conclusions, and final approval of the manuscript. All authors have critically reviewed and approved the final draft and are responsible for the content and similarity index of the manuscript.

Conflicts of interest disclosure

The authors have no conflict of interest to declare.

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Guarantor

Jeza M.Abdul Aziz is the Guarantor of the submission.

Data availability statement

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Not commissioned, externally peer-reviewed.