Eagle Syndrome Research Articles

Ischemic stroke due to stylocarotid artery syndrome: a case report and review.

Stylocarotid artery syndrome (SAS) is a rare variant of Eagle's syndrome that may lead to transient ischemic attack or stroke. The underlying pathophysiological mechanism involves compression of the internal carotid artery by an elongated styloid process (ESP), potentially resulting in vascular occlusion or dissection. An ESP exceeding 2.5 cm is deemed elongated, with a length of 3.0 cm considered clinically significant. Although the prevalence of ESP ranges from 4.0% to 7.3%, symptomatic cases are rare; symptoms are present in only approximately 4.0% of individuals with an ESP. Unlike the typical symptoms of Eagle's syndrome, SAS may not cause pharyngeal discomfort, the sensation of a foreign body in the throat, dysphagia, or facial pain. This absence of characteristic symptoms as well as the development of central nervous system symptoms often leads patients to seek care from neurologists instead of otolaryngologists, increasing the likelihood of misdiagnosis or underdiagnosis. We herein report a unique case of ischemic stroke caused by SAS and present a literature review on cases of SAS-associated ischemic stroke published in the past decade. The reporting of this study conforms to the CARE guidelines.

Transoral Robotic Surgery for Eagle Syndrome: A Systematic Review.

Objectives: Transoral robotic surgery (TORS) is gaining popularity and has been introduced for the treatment of Eagle syndrome. This review aims to evaluate the safety and efficacy of TORS for the treatment of Eagle syndrome. Methods: A systematic review of the English language literature using multiple databases was completed for studies describing TORS for Eagle syndrome. The quality of studies and risk of bias were evaluated using the MINORS scoring system. Results: Out of 1495 articles screened, 4 studies met criteria for inclusion in the final analysis. Across all studies, there was a 100% surgical success rate. In total, every patient had some level of symptom improvement with 84% of patients having complete symptom improvement and 16% having partial improvement. Reported estimated blood loss averaged 12.5 mL. A total of 94.7% of patients had no surgical complications and no cases were complicated by postoperative bleeding. Operative time averaged 65 minutes. The average length of stay was 2.1 days. A total of 92% of patients resumed their diet on postoperative day 1, with the remainder resuming on postoperative day 2. MINORS criteria scoring suggested moderate risk of bias in all studies. Conclusion: Based on limited quality evidence, this review suggests that TORS is a safe and effective surgical approach in the treatment of Eagle syndrome with low complication rates. Further large-scale prospective studies are warranted.

Adaptive treatment strategy for a vestibular schwannoma in a patient with vascular Eagle syndrome: illustrative case.

Eagle syndrome, an uncommon condition, causes symptoms due to neural and/or vascular compression from an elongated styloid process or calcified stylohyoid ligament and can also complicate other planned surgical procedures. A 42-year-old female with loss of balance, dizziness, and ataxic gait underwent cranial magnetic resonance imaging (MRI), revealing a right-sided Koos grade IV vestibular schwannoma. Initially, a retrosigmoid craniotomy for tumor resection was planned. However, preoperative MRI and computed tomography (CT) showed a dilated right-sided mastoid emissary vein, tortuous scalp and paraspinal veins, and bilateral elongated styloid processes. CT angiography and digital subtraction angiography indicated Eagle syndrome-related compression of both internal jugular veins and concurrent occlusion of the left internal jugular vein at the jugular foramen. Consequently, given the risk of damaging venous structures, Gamma Knife radiosurgery was chosen over resection. This case highlights the importance of adapting treatment plans based on patient-specific anatomical and pathological factors. In situations in which traditional surgery poses risks to sensitive structures such as the venous system, alternative approaches like radiosurgery offer safer yet effective options. Comprehensive risk-benefit evaluations are crucial for such decisions.

Eagle Syndrome (Calcification of the Stylohyoid Ligament) Excision

Eagle Syndrome (Calcification of the Stylohyoid Ligament) Excision

Diagnosis and treatment of Eagle's syndrome and possible complications.

Eagle's syndrome (ES) is a term describing a group of symptoms associated with the elongation of the styloid process (SP) of the temporal bone or with ossification of the stylohyoid ligament. Clinically, it manifests through pain in the orofacial and pharyngeal regions, similar to that experienced by patients with temporomandibular joint disease. The presented paper aims to assess the evaluation of the success of surgical treatment and the complications associated with such treatment. Our retrospective study includes nine patients with ES treated in our Department Oral and Maxillofacial Surgery in years 2019-2023 (median age of 47 years) - all patients affected with this disease out of 2,716 patients examined at our department due to a temporomandibular joint disease. Eight of these patients underwent a surgical resection of the SP. The success and complications in these patients are evaluated in this study. In seven patients, the problems disappeared or at least diminished, which is consistent with data from the literature. Postoperative complications such as deficit of the marginal branch of the facial nerve (n. VII), limited sticking the tongue out (n. XII), and velopharyngeal insufficiency (n. IX) occurred in three patients. The wide spectrum of clinical manifestations makes ES a condition that should be considered in the differential diagnosis of temporomandibular joint disorders, post-traumatic conditions, or complicated eruption of wisdom teeth or their extraction. ES can be treated conservatively or (mostly) surgically. Surgical treatment can be relatively successful and effective, but it is necessary to respect the anatomical placement of the SP, which can be associated with the development of complications, especially in cases with extremely long SP.

Elongated styloid process of an autopsied skull from the Cemetery of Santa Maria Maggiore in Vercelli, 18th-19th century (Piedmont, northern Italy).

We discuss the coexistence of a postmortem cut and a pathological alteration, recorded on a skeleton belonging to an adult man that was discovered during the archaeological investigations of the cemetery of the Church of Santa Maria Maggiore in Vercelli (northern Italy, 18th-19th century). The skull presents an oblique cleft, which from the top of the frontal bone bends towards the occipital, and the left styloid process is elongated compared to normal values (48 mm). The elongated styloid process is due to the ossification of the styloid ligament which has several possible causes. To increase the knowledge about this pathological condition in the past, it was necessary to compare all the data present in the literature today and consider the few cases published in the paleopathological field. In this paper, our main goals are: i) to investigate the reasons for which the craniotomy was performed; ii) to examine the possible cause of the ossification of the styloid process, described as Eagle's syndrome; iii) to enrich the archaeological literature of elongated styloid process cases and iv) to investigate the presence of a hypothetical relationship between the autopsy cut and the diagnosed Eagle's syndrome on this skull.

Role of the Surgical Approach in the Treatment of Eagle Syndrome.

Introduction Eagle syndrome is a rare and an often misdiagnosed entity in otorhinolaryngology. Objective To determine the efficacy of the surgical treatment for Eagle syndrome. Methods The present prospective study included 25 patients who presented with complaints of pain in the throat, ear, and neck, as well as difficulty and/or pain while swallowing; they were assessed for Eagle syndrome. As per patient profile, we performed a clinical assessments along with orthopantomograms (OPGs), three-dimensional computed tomography (3D CT) scans, and cone beam computed tomography (CBCT). Pain was assessed pre- and postoperatively through the Numerical Rating Scale-11 (NRS-11), whose score ranges from 0 to 10. Microscopic tonsillo-styloidectomy was performed in cases in which the conservative treatment failed to relieve pain. Results The mean age of the entire study population was of 36.08 ± 7.19 years, and the male-to-female ratio was of 1.08:1. Referred otalgia was the commonest (44%) complaint. Radiologically, out of 25 patients, 20 patients presented elongated styloid processes. The longest symptomatic styloid process measured radiographically was of 64.7 mm while the shortest was of 28.2 mm. Out of 20 patients, 12 underwent surgery. The postoperative pain assessment through the NRS-11 was performed on day 0 (3.83 ± 0.83), day 7 (1.5 ± 0.52), week 4 (0.5 ± 0.52), and week 12 (0.41 ± 0.51). By 12 weeks, 7 patients were symptom-free, while 5 patients still reported mild pain. Conclusion Eagle syndrome associated with an elongated styloid process is not a rarity, but it often goes undiagnosed. Microscopic tonsillo-styloidectomy shows excellent results in the management of patients with Eagle syndrome.

Surgical management of stylocarotid Eagle syndrome in a patient with bilateral internal carotid artery dissection: illustrative case.

Eagle syndrome is characterized by an elongated styloid process, which can cause acute neurological symptoms when the projection impinges on local structures. One method by which Eagle syndrome can cause acute stroke is via internal carotid artery dissection. A patient presented with acute aphasia and right-arm weakness. Imaging revealed a left internal carotid artery dissection, which was treated with stenting. Three years later, the patient presented with left-sided weakness, and imaging revealed a new right internal carotid artery dissection. Closer review of the patient's imaging revealed bilateral elongated styloid processes. The patient subsequently underwent staged bilateral styloidectomy and returned to his prior baseline postoperatively. This case report describes a patient with Eagle syndrome who had two internal carotid artery dissections separated by several years. A literature review revealed that styloidectomy is well tolerated in patients with carotid dissection due to Eagle syndrome. Patients with carotid dissection due to Eagle syndrome remain at risk for contralateral dissection, and prophylactic contralateral styloidectomy should be considered.

Retraction: Eagle Syndrome: A Rare Cause of Stroke in a Young Patient.

[This retracts the article DOI: 10.7759/cureus.21102.].

Clinical implications of the styloid process elongation: what the dental practitioner should know

The styloid process (SP) is a subtle osseous projection that arises from the undersurface of the temporal bone. It forms, with the styloid ligament and the lesser cornu of the hyoid bone, the stylohyoid chain. SP offers attachment to different muscles and ligaments and is surrounded by major nerves and vessels. Elongation of the SP is an uncommon condition. Some patients with elongated SP may experience a set of symptoms referred to as Eagle syndrome. The latter symptoms develop as a consequence of an aberrant SP irritating nearby anatomic structures. Eagle syndrome diagnosis is challenging; various imaging techniques are helpful in identifying the SP elongation, among them panoramic radiographs and three-dimensional images like CT and CBCT scans, which allow for better visualization. Depending on the severity, surgical or conservative treatment options are available for Eagle syndrome. However, it is advised to start out conservatively.

Accuracy in the Diagnostics of Styloid Process - Panoramic Radiograph vs. Computed Tomography: A Comparative Study.

The styloid process (SP) becomes clinically relevant when it shows enlargement (>30 mm) in the sense of an elongated SP (ESP) and/or increasing calcification leading to Eagle Syndrome (ES). Panoramic radiograph (PR) or computed tomography (CT) are part of the routine diagnostics in ES. Currently, CT is considered the gold standard. The aim of this study was to investigate the accuracy in the diagnostics/measurements of SP/ESP throughout a comparative study between PR and CT. Furthermore, in addition to measuring established parameters, this study aimed to determine the currently unexamined width in the base and tip of the SP. The present study examined the radiological findings of bilateral SP in 100 patients who received both PR and CT on the same day. Measurements of the length of the SP and width at the basis and tip were performed. Furthermore, calcification patterns, Langlais classification and the prevalence of ESP were analyzed. There was a highly significant correlation between PR and CT measuring SP for every parameter. Males showed significantly longer SP than females among the age group between 18-75 years. The results of the length measurements of the SP (male: right SP=32.98 mm; left SP=35.21 mm; female: right SP=30.31 mm; left SP=30.92 mm) significantly exceeded the values of comparable studies. Consequently, it can be concluded that PR provides accurate measurements when compared to CT for measuring and diagnosing SP/ESP/Eagle syndrome. This study was one of the first to examine the width of the SP in the base and tip, thus these measurements can serve as a baseline for further studies. Since the mean lengths of SP exceeded 30.0 mm in the present study, these findings raise the question of whether the cut-off of 30.0 mm is adequate for the diagnosis of ESP.

Case of elongated styloid process detected in forensic medical examination of skeletal remains

There are cases of skeletal developmental features that have high identification significance for the search of missing person in forensic medical practice of skeletal remains examination. The features of the skull's anatomical structure may indicate the presence of some clinical syndromes, for which a person could have sought medical attention in the lifetime. In the described case, elongated styloid appendages of the temporal bones were identified on the skull of an unknown person found within the territory of the Leningrad region, which could be a sign of the stylohyoid syndrome (Eagle syndrome). Various clinical picture could be observed depending on the size and degree of styloid processes. The objective of the forensic medical expert was to establish the probability of occurrence of the stylohyoid syndrome and its possible clinical signs.

Eagle’s syndrome. A clinical case

Eagle syndrome represents the set of symptoms caused by the elongation of styloid process. This can trigger the irritation of neuro–vascular structures near its vicinity, for example the glossopharyngeal nerve that can lead to deglutition disorders, dysphagia, globus sensation, neck and facial pain, headache. It is a diagnosis of exclusion and needs a high level of clinical suspicion being a rare condition without reports of incidence in the population of Moldova. In this article we present a case of bilateral elongation of styloid processes that caused persistent neck and facial pain in a patient that does not respond to analgetic drugs. The patient underwent unilateral styloidectomy by cervical approach, which resulted for the symptoms to disappear.

Artificial Intelligence: A Reliable Tool to Detect the Elongation of the Styloid Process.

Background Eagle's syndrome is characterized by the anomalous elongation of the styloid process. This condition is usually identified through the manual evaluation of orthopantomogram (OPG) images, which is time-consuming and can have interobserver variability. The application of Artificial intelligence (AI) in radiology is gaining importance and interest in recent years. The application of AI in detecting styloid process elongation is less explored, advocating for research in the same arena. Aim and objectives The study aimed to evaluate the accuracy of artificial intelligence in detecting styloid process elongation in digital OPGs and to compare the performance of the three different AI algorithms with that of the manual radiographic evaluation by the radiologist. Materials and methods A total of 400 digital OPGs were screened, and linear measurements of the styloid process length (ImageJ software (National Institute of Health, Maryland, USA)) were done for the identification of styloid process elongation by a single calibrated observer to finally include a processed image dataset including 169 images of the elongated styloid process and 200 images of the normal styloid process.A machine learning approach was used to detect the styloid process elongation using the three different AI models: logistic regression, neural network, and Naïve Bayes algorithms in Orange software (University of Ljubljana, Slovenia). Performance evaluation was done using the accuracy, sensitivity, specificity, precision, recall, F1 score, and AUC-ROC (area under the receiver operating characteristic) curve. Results Logistic regression and neural network algorithms depicted the highest accuracy of 100% with no false positives or false negatives, securing a score of 1.000 for all the metrics.However, the Naïve Bayes model demonstrated a fairly considerable accuracy, classifying 49 false positive images and 59 false negative images with an AUC (area under the curve) score of 78 %. Nevertheless, it performed better than random guessing. Conclusion Logistic regression and neural network algorithms accurately detected styloid process elongation similar to that of manual radiographic evaluation. The Naïve Bayes algorithm did not perform an accurate classification yet performed better than random guessing. AI holds a promising scope for its application in automatically detecting styloid process elongation in digital OPGs.

When Eagle Stares into The Eye

Retinal pigment epithelial detachment (PED) is the separation of the retinal pigment epithelium(RPE) from the Bruch’s membrane(BM). Eagle syndrome(ES) is characterized by an abnormally elongated styloid process with/without abnormal direction and/or ossification of the styloid ligament. Presence of both the above entities together is a rarest of rare sight. It may be a coincidence, or the diseases may have an association. Hence, further studies are warranted.

Eagle syndrome: An updated review.

This work aims to review the current literature and our experience on vascular Eagle syndrome (ES) that can present misleading clinical presentations and better understand the possible therapeutic strategies. We reviewed the existing literature on PubMed from January 1, 2017, to December 31, 2022, including the sequential keywords "vascular AND Eagle syndrome," "vascular AND styloid syndrome," "vascular AND elongated styloid process," "vascular AND stylocarotid syndrome," and "Eagle syndrome AND carotid artery dissection." 38 vascular ES cases, including our experience, were analyzed. The most frequent clinical onset was hemiparesis (n 21, 57%), but other regular clinical presentations were aphasia, loss of consciousness, amaurosis, headache, or a combination of the latter. Massive oral bleeding was reported only once in the literature before our case. Twelve patients were treated with only antiplatelet therapy, either single or double. Nine patients were treated with anticoagulation therapy only. In 14 patients, a carotid artery stent was used, associated with anticoagulation or antiplatelet therapy. In 17 cases, a styloid process (SP) resection was performed. ES has many clinical presentations, and carotid artery dissection resulting in oral bleeding seems rare. Literature results and our experience make us believe that when dealing with vascular ES, the best treatment strategy is endovascular internal carotid artery stenting with antiplatelet therapy, followed by surgical removal of the elongated SP to prevent stent fracture.

Bilateral elongated styloid process: report of a dry skull

Temporal styloid process is a thin, fragile, pointed bony projection directed forward and downward with average length of 2 to 2.5 cm. The authors describe a skull with two enlarged styloid processes, found in the osteotheque of the Faculty of Medicine of the Eduardo Mondlane University. The objective of this presentation is to draw the attention of the medical community to the existence of these situations that in real life give different and non-specific symptoms that can make one think of another diagnosis. The clinical picture of Eagle's Syndrome ranges from asymptomatic cases to cases with great discomfort, with variable and non-specific symptoms, due to its close relationship with the vasculonervous bundle of the neck and other cranial nerves and its knowledge is important for the student of anatomy, anatomist, general practitioner, dentist, radiologist, neurologist, maxillofacial, neurosurgery, otolaryngologist and psychiatry.

Minimally Invasive Cervical Styloidectomy in Stylohyoid Syndrome (Eagle Syndrome).

(1) Background: Stylohyoid syndrome, known as classical Eagle Syndrome (cES), is characterized by calcification of the stylohyoid chain with numerous nonspecific symptoms, mainly pain. This study introduces minimally invasive cervical styloidectomy (MICS). (2) Methods: MICS was performed on sixty-five patients diagnosed with classical Eagle Syndrome. Patients underwent meticulous differential diagnosis. Surgical plans were established based on the findings from neck angioCT. (3) Results: The healing process was uneventful, without significant complications. The overall success rate was 97.0%, with a follow-up of a minimum of six months. In one case, the surgery did not yield the desired improvement. In one case, a partial relapse of symptoms was observed. (4) Conclusions: MICS is a straightforward and efficient surgical treatment technique for stylohyoid syndrome.

Neurosurgical implications of the Jugular Vein Nutcracker

In the last ten years, a new variant of Eagle Syndrome is emerging and being described: Styloid Jugular Nutcracker (SJN). In SJN, an elongated or vertically directed styloid process causes jugular vein stenosis by compressing the vein against the arch of C1. The clinical consequences appear to be various and misunderstood, ascribable mainly to venous flow impairment and consequent intracranial hypertension. The aim of this paper is to create an overview of Jugular Vein Nutcracker and to focus on its neurosurgical implications. A PRISMA-based literature search was performed to select the most relevant papers on the topic and to realize a mini-review. Future searches in the neurosurgical field should focus on collecting data about further causes of jugular stenosis compression and the association of SJN with cerebrovascular diseases. It would also be interesting to investigate the potential role of primary and secondary prevention, which is unknown so far.

Eagle's syndrome mimicking thyroid-associated orbitopathy.

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