A Prolonged Time to Diagnosis Due to Misdiagnoses: A Case Report of an Atypical Presentation of Eagle Syndrome

Abstract

Patient: Male, 23-year-old Final Diagnosis: Eagle syndrome Symptoms: Pain Medication: — Clinical Procedure: — Specialty: Dentistry • Pathology Objective: Unusual clinical course Background: Eagle syndrome is an unusual condition in which the styloid process (SP) becomes elongated and causes different clinical symptoms due to pressure on adjacent anatomical structures. The symptoms are non-specific and include severe throat, facial, and ear pain, or headaches. They are usually exacerbated by head rotation, swallowing, yawning, or chewing, but atypical presentations exist. It is a difficult pathology to diagnose and it can take several years before a proper diagnosis is made. Case Report: This report describes the case of a dental student presenting with an atypical presentation of Eagle syndrome. His styloid processes were 75 mm long and he was affected with severe pain to his throat, the anterior part of his ears, his submandibular area, and his molar teeth. The pain was exacerbated during maximal mouth opening, yawning, mandibular protrusion, and during downward head tilt, but not during the classically described movements of head rotation, swallowing, yawning, or chewing. Due to the absence of the classic symptoms, even with knowledge of the condition and unusual direct access to several oral and maxillofacial specialists, it took 4 years and multiple misdiagnoses to reach the final diagnosis. Following bilateral styloidectomy, all pain subsided. Conclusions: The clinical presentation of Eagle syndrome varies, and the symptoms are non-specific. This greatly increases the complexity of diagnosing the condition and raises the time to diagnosis and the chances of misdiagnoses. It is, therefore, crucial to recognize the diagnostic tools, applicable imaging, and definitive treatment alternatives to successfully identify and treat patients affected.

Eagle’s Syndrome: An Unusual cause of throat pain-Case Study

“…1 Eagle's syndrome is often misdiagnosed due to its rarity and non-specific symptoms, and delay to diagnosis has been reported to be between 10 and 27 years. 1,3,4 In our case report, it took almost an year for the patient to be diagnosed correctly. Similarly, it has been reported that despite that the patient having knowledge of Eagle's syndrome, it took 4 years to reach the correct diagnosis due to the unusual presentation of his symptoms.…”

“…Similarly, it has been reported that despite that the patient having knowledge of Eagle's syndrome, it took 4 years to reach the correct diagnosis due to the unusual presentation of his symptoms. 3 The lack of a clear aetiological link and the lack of knowledge about this clinical entity contribute to misdiagnosis. 1 Patients with severe calcification of the SP have also been reported to be asymptomatic.…”

“…2 Non-invasive treatment options include analgesic/opioid medications, steroid injections, non-steroidal antiinflammatory agents, oral antidepressants, anticonvulsants, and local anesthetic agents. 3,5 Surgical styloidectomy has been reported to have an 80% cure rate. 1 Eagle first described an easy, quick, and less time-consuming intraoral or transoral approach to the styloid process 1,3 However, the final choice to proceed with surgery is contentious, and there is no prevailing opinion in the favour of a surgical approach, and the patient must be managed according to the presenting symptoms 5 The symptoms of our patient had not been alleviated despite administration of painkillers, as a result, we chose surgical treatment.…”

“…An aggregate of symptoms caused by the elongation and severe calcification of the temporal bone's styloid process, as well as calcification of the ligaments attached to this process, including the styloid and stylomandibular ligaments, have been identified 2 . The styloid process is typically 20 to 30 mm in length, and patients with elongated styloid processes of 40 mm or longer have clinical distress 1,3 . This styloid process elongation could be unilateral or bilateral 4 .…”

“…This styloid process elongation could be unilateral or bilateral 4 . It affects around 4% of the general population, with 3:1 female predominance, however only 4% of these people experience associated symptoms 3 . Eagle mainly characterized two syndromes: "classic styloid syndrome" following tonsillectomy, and "stylocarotid syndrome" unrelated to tonsillectomy 6 .…”