Carotid Artery Dissection Caused by an Elongated Styloid Process: A Case Report

Article information

J Neurosonol Neuroimag. 2024;16(1):21-23
Publication date (electronic) : 2024 June 30
doi : https://doi.org/10.31728/jnn.2024.00152
*Stroke Center, Pusan National University Hospital, School of Medicine, Pusan National University, Busan, Korea
Department of Neurology, Pusan National University Hospital, School of Medicine, Pusan National University, Busan, Korea
Biomedical Research Institute, Pusan National University Hospital, School of Medicine, Pusan National University, Busan, Korea
§Department of Radiology, Pusan National University Hospital, School of Medicine, Pusan National University, Busan, Korea
Correspondence: Sang Min Sung, MD, PhD Stroke Center, Department of Neurology, Biomedical Research Institute, Pusan National University Hospital, School of Medicine, Pusan National University, 179 Gudeok-ro, Seo-gu, Busan 49241, Korea Tel: +82-51-240-7317 Fax: +82-51-245-2783 E-mail: aminoff@hanmail.net
Received 2024 April 15; Revised 2024 May 24; Accepted 2024 June 9.

Abstract

Eagle’s syndrome is caused by an elongated styloid process or calcified stylohyoid ligament that interferes with adjacent structures. Herein, we present the case of a 26-year-old man who presented to the emergency department due to sudden onset pain in the right occipital area, dysarthria, left hemiparesis, and left arm paresthesia. Computed tomography (CT) angiography revealed an intimal flap and thrombus in the proximal right internal carotid artery (ICA), indicative of carotid artery dissection, as well as direct contact between the styloid process and ICA on both sides. The patient underwent anticoagulation with intravenous heparin for 7 days, followed by 75 mg of clopidogrel per day. Following treatment, the patient gradually improved and was discharged with slight paresthesia in the left arm two weeks after symptom onset. Carotid artery dissection is a rare manifestation of Eagle’s syndrome caused by an elongated styloid process.

Eagle’s syndrome, which may be associated with various symptoms, is caused by an elongated styloid process or calcified stylohyoid ligament that interferes with adjacent structures. The otolaryngologist Watt W. Eagle, who first described the clinical findings of Eagle’s syndrome in 1937, classified it into two types. The first and more common type, termed ‘classic’ eagle syndrome, occurs after tonsillectomy or other pharyngeal surgeries, presenting with unilateral facial or neck pain, otalgia, sore throat, dysphagia, tinnitus, and foreign body sensation in the throat. The second type, stylocarotid artery syndrome, may present as a transient ischemic attack or stroke resulting from impingement of an elongated styloid process on the carotid artery.1,2 Carotid artery dissection is an uncommon complication of stylocarotid artery syndrome; due to its rarity, there is no consensus regarding the optimal management of this condition. Herein, we report a case of internal carotid artery dissection caused by impingement of the styloid process with elongation and proximity to the carotid artery.

CASE

A 26-year-old man presented to the emergency department due to sudden onset of pain in the right occipital area, dysarthria, left hemiparesis, and left arm paresthesia. His National Institutes of Health Stroke Scale (NIHSS) at admission was 3. His medical history was unremarkable, except for a 5-year history of cigarette smoking and a habit of repetitively turning his head from side to side due to neck discomfort. Diffusion-weighted brain magnetic resonance imaging revealed an acute multifocal infarction in the border zone between the right middle and anterior cerebral arteries (Fig. 1A). Initial computed tomography (CT) angiography further revealed an intimal flap and thrombus in the proximal right internal carotid artery (ICA), suggestive of carotid artery dissection, in addition to direct contact between the styloid process and the ICA on both sides (Fig. 1B, C). Additionally, the bilateral styloid processes were found to be elongated, measuring 39.2 mm and 31.2 mm on the right and left sides, respectively. Right common carotid angiogram confirmed acute dissection of the right ICA with an intimal flap and thrombus in the false lumen (Fig. 1D). We initially planned to perform endovascular treatment with a stent for carotid artery dissection, but the patient refused endovascular stenting. Thereafter, the patient was anticoagulated with intravenous heparin for 7 days, followed by 75 mg of clopidogrel per day. The patient’s condition gradually improved following treatment, and he was discharged with slight paresthesia in the left arm two weeks after symptom onset. We discussed surgical excision of the styloid process with an otorhinology specialist, and decided to withhold the operation unless the patient had new symptoms. The patient avoided excessive head bending and remained neurologically intact at the 4-year follow-up visit.

Fig. 1.

(A) Diffusion-weighted imaging showing multifocal acute infarction in the right MCA-ACA border zone area. Axial (B) and coronal (C) CT angiograms showing direct contact between the elongated styloid process (black arrow) and dissected cervical ICA (white arrow). (D) Right common carotid angiogram showing an increased external diameter, intimal flap, and false and true lumen of the cervical ICA. MCA-ACA, OOO; CT, computed tomography; ICA, internal carotid artery.

DISCUSSION

The styloid process is a bony structure projecting from the temporal bone which is anatomically adjacent to the internal and external carotid arteries, internal jugular vein, and the cranial nerves V, IX, X, and XII. Although anatomical variations in the styloid process have been observed in the general population, a styloid process longer than 30 mm is generally defined as elongated, and is considered to be responsible for the symptoms of eagle syndrome.3 Additional anatomical factors of the styloid process, including its proximity to the carotid artery, angulation, and width, may contribute to the occurrence of the syndrome. Previous studies have further shown that carotid artery dissection, a rare complication of stylocarotid artery syndrome, is statistically associated with a styloid process which is elongated or exceptionally close to the carotid artery.4,5 CT with three-dimensional reformations is a valuable imaging tool that accurately demonstrates the anatomy of the styloid process and its adjacent structures.2,3 Eagle’s syndrome can be managed conservatively or surgically based on symptom severity.3,5 To date, no definite guidelines have yet been established for the treatment of stylocarotid artery syndrome with a secondarily dissected carotid artery. Recanalization therapies, such as endovascular treatment, may be considered according to the management guidelines for acute ischemic stroke. Furthermore, surgical excision of the elongated styloid process, using an intraoral or extraoral approach, may be necessary to prevent recurrences.3 In the present case, the patient improved after medical treatment and remained symptom-free at the 4-year follow-up visit. Carotid artery dissection is a rare manifestation of Eagle’s syndrome caused by an elongated styloid process. Conservative medical treatment can be applied for the initial management and education to avoid excessive neck turning.

Notes

Ethics Statement

Written informed consent was obtained from the patient to report demographic data, medical conditions, neuroimaging, and treatment.

Availability of Data and Material

The datasets generated or analyzed during the study are available from the corresponding author upon reasonable request.

Author Contributions

All authors have read and approved the manuscript. Writing the initial draft of the manuscript: SMS, YJK. Conceptualization and supervision: SMS, YJK. Medical management of the case: SMS, YJK, THL. Critically revising the manuscript and literature review: YJK, SMS, HJC, THL.

Sources of Funding

This work was supported by clinical research grant from Pusan National University Hospital in 2024.

Conflicts of Interest

No potential conflicts of interest relevant to this article was reported.

Acknowledgements

None.

References

1. Eagle WW. Elongated styloid processes: report of two cases. Arch Otolaryngol 1937;25:584–587.
2. David J, Lieb M, Rahimi SA. Stylocarotid artery syndrome. J Vasc Surg 2014;60:1661–1663.
3. Piagkou M, Anagnostopoulou S, Kouladouros K, Piagkos G. Eagle’s syndrome: a review of the literature. Clin Anat 2009;22:545–558.
4. Razak A, Short JL, Hussain SI. Carotid artery dissection due to elongated styloid process: a self-stabbing phenomenon. J Neuroimaging 2014;24:298–301.
5. Taheri A, Firouzi-Marani S, Khoshbin M. Nonsurgical treatment of stylohyoid (Eagle) syndrome: a case report. J Korean Assoc Oral Maxillofac Surg 2014;40:246–249.

Article information Continued

Fig. 1.

(A) Diffusion-weighted imaging showing multifocal acute infarction in the right MCA-ACA border zone area. Axial (B) and coronal (C) CT angiograms showing direct contact between the elongated styloid process (black arrow) and dissected cervical ICA (white arrow). (D) Right common carotid angiogram showing an increased external diameter, intimal flap, and false and true lumen of the cervical ICA. MCA-ACA, OOO; CT, computed tomography; ICA, internal carotid artery.