Eagle Syndrome and Internal Carotid Artery Dissection: Description of Five Cases Treated in Two Italian Institutions and Review of the Literature.

Eagle syndrome is a rare pattern of symptoms (0.16% of the general population) due to the conflict with adjacent anatomical structures by an elongated styloid process or a calcified stylohyoid ligament; 2 variants of this condition have been described in the literature, classical and vascular. The classical form is caused by compression of the glossopharyngeal nerve and the surrounding structures from an abnormal stylohyoid apparatus, causing odynophagia and neck pain and is usually treated by an otorhinolaryngologist. The vascular form, determined by the conflict between the osteoligamentous malformation and the extracranial carotid artery, can cause neurological symptoms due to the compression of the vessel or in some cases the dissection of the carotid artery itself. However, an elongated styloid process occurs in about 4% of the general population, and the most recent literature shows that the vascular form of Eagle syndrome could be an underestimated cause of carotid artery dissection (CAD) and should be considered in the differential diagnosis of this condition. In addition to the literature many different treatment options for this condition are reported, either medical and/or surgical, but an ideal approach has not yet been fully identified. We report 5 cases of internal CAD due to the vascular variant of Eagle syndrome treated in 2 different Italian institutions (Department of Vascular and Endovascular Surgery, Galliera Hospital, Genoa and Department of Vascular Surgery, Santi Filippo e Nicola Hospital, Avezzano, L'Aquila) and a careful and analytical review of the available literature on this topic.

Surgical management of Eagle syndrome: A 17-year experience with open and transoral robotic styloidectomy.

Eagle Syndrome (ES) is a rare disorder that can present with symptoms ranging from globus sensation to otalgia that is attributed to an elongated styloid process and/or calcified stylohyoid ligament. No standardized treatment algorithm exists, and although various surgical approaches have been described, data on the use of transoral robotic surgery (TORS) in this population is limited. To investigate the utility of TORS in the treatment of ES, a retrospective review in 19 ES patients was carried out at a single academic, tertiary medical center between 2000 and 2017. Nineteen patients underwent twenty-one styloid resections: 6 performed via TORS and 15 via transcervical approach. Across all patients, 90% reported some degree of lasting improvement in symptoms while 55% reported significant improvement. When TORS was compared to transcervical resection, there was no difference in the subjective rate of "meaningful" (83 vs. 57%) versus rate of "non-meaningful" symptom improvement (17 vs. 43%) (p = 0.35). There was a trend towards less estimated blood loss (EBL), operative time, and post-operative length of stay (LOS) with TORS versus transcervical cases (9.2 mL vs. 30.0 mL, 98 vs. 156 min, and 0.7 vs. 1.2 days); however, these did not reach statistical significance (p = .11, 0.13, and 0.42, respectively). Three patients experienced complications associated with an open approach, as compared to none with TORS. In select patients, TORS styloidectomy is a reasonable surgical alternative to traditional transoral and transcervical techniques as it provides similar symptom improvement, and reduced length of stay, blood loss, and operative time.

Stylocarotid artery syndrome.

Stylocarotid artery syndrome is a rare condition that results from compression of the internal or external carotid artery by the styloid process of the temporal bone. Here we present the case of a patient suffering from syncope, monoparesis of the right arm, and dysarthria due to recurrent transient ischemic attacks that resulted from severe compression of the midsegment of the left extracranial internal carotid artery between an elongated styloid process and a C2 vertebral body osteophyte. This case demonstrates successful surgical management of a condition rarely encountered by the vascular surgeon.

Neurological phenotypes and treatment outcomes in Eagle syndrome: systematic review and meta-analysis.

Background: Eagle syndrome is caused by an elongated styloid process affecting carotid arteries and cranial nerves. Pain, dysphagia, tinnitus, paresthesia (classic subtype), and neurovascular events (vascular subtype) may be triggered by head movements or arise spontaneously. However, Eagle syndrome remains underappreciated in the neurological community. We aimed to determine the most common neurological and non-neurological clinical presentations in patients with Eagle syndrome and to assess the clinical outcome post-surgical resection in comparison to non-surgical therapies. Methodology: We conducted a systematic review of patient-level data on adults with Eagle syndrome, following PRISMA guidelines. We extracted data on demographics, presenting symptoms, neurological deficits, radiological findings, and treatments, including outcomes and complications, from studies in multiple indexing databases published between 2000 and 2023. The study protocol is registered with PROSPERO. Results: In total, 285 studies met inclusion criteria, including 497 patients with Eagle syndrome (mean age 47.3 years; 49.8% female). Classical Eagle (370 patients, 74.5%) was more frequent than vascular Eagle syndrome (117 patients, 23.5%, p < 0.0001). Six patients (1.2%) presented with both variants and the subvariant for four patients (0.8%) was unknown. There was a male preponderance (70.1% male) in the vascular subtype. A history of tonsillectomy was more frequent in classic (48/153 cases) than in vascular (2/33 cases) Eagle syndrome (Odds Ratio 5.2, 95% CI [1.2-22.4]; p = 0.028). By contrast, cervical movements as trigger factors were more prevalent in vascular (12/33 cases) than in classic (7/153 cases) Eagle syndrome (Odds Ratio 7.95, 95% CI [2.9-21.7]; p = 0.0001). Headache and Horner syndrome were more frequent in vascular Eagle syndrome and dysphagia and neck pain more prominent in classic Eagle syndrome (all p < 0.01). Surgically treated patients achieved overall better outcomes than medically treated ones: Eighty-one (65.9%) of 123 medically treated patients experienced improvement or complete resolution, while the same applied to 313 (97.8%) of 320 surgical patients (Odds Ratio 1.49, 95% CI [1.1-2.0]; p = 0.016). Conclusions: Eagle syndrome is underdiagnosed with potentially serious neurovascular complications, including ischemic stroke. Surgical treatment achieves better outcomes than conservative management. Although traditionally the domain of otorhinolaryngologist, neurologist should include this syndrome in differential diagnostic considerations because of the varied neurological presentations that are amenable to effective treatment.

[Eagle's syndrome: Presentation of a case in the Primary Care clinic]. / Síndrome de Eagle. Presentación de un caso en la consulta de atención primaria.

Eagle's syndrome is rare condition described in 1937 by W. Eagle, and is secondary to the elongation of the styloid process and/or calcification of the stylohyoid ligament. Although it occurs asymptomatically in the majority of cases, the pressure exerted by this alternative structure from an anatomical point of view against the neighbouring areas can trigger a great variety of symptoms. It is often wrongly diagnosed, leading to multiple interconsultations with different professionals. This means that the professional must take it into account in those cases of pain in the maxillofacial region without any clear aetiology. The case is presented of a 63year-old patient in whom the only symptom was an invalidating occipital neuralgia, with visits to numerous physicians. A 3-dimension cone beam computed tomography (CBCT) confirmed the clinical suspicion. The treatment consisted of partial resection of both styloid processes.

Systematic review of cochlear implantation in CHARGE syndrome.

Objective: CHARGE syndrome presents with a collection of congenital anomalies affecting multiple organs. Ear and temporal bone anomalies, including hearing loss are highly prevalent. We present an aid to management of this challenging condition and report the strategies and outcomes of cochlear implantation. Methods: Systematic review of Medline, EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to 21/10/2018 The review and meta-analysis of studies were performed according to the PRISMA statement. Patient demographics, comorbidity, anatomical factors, details of cochlear implantation and audiological outcome were extracted and summarized. Anatomical and surgical factors were evaluated by meta-analysis. Audiological outcomes reported were too heterogeneous for meta-analysis. All statistics were calculated with SPSS v23.0 (IBM, New York, USA). Results: Thirty-one studies reported 165 cochlear implants in 156 patients with CHARGE syndrome. Temporal bone and facial nerve anomalies were common. Discussion: The assessment and management of patients with CHARGE syndrome undergoing cochlear implantation is challenging. Outcomes may be affected by cochlear nerve deficiency, inner ear anomalies, and developmental delay. Surgery is almost invariably complicated by abnormal anatomy, and increased incidence of complications. Conclusion: A careful, case-by-case assessment of an individual's requirements within a multi-disciplinary setup is essential for achieving the best possible outcome.

Aspectos clínicos, radiográficos e estratégias terapêuticas da Síndrome de Eagle: revisão de literatura / Clinical, radiographic aspects and therapeutic strategies of Eagle Syndrome: literature review

Objetivo: Este trabalho tem como propósito fornecer uma análise abrangente das características anatômicas, clínicas e radiográficas da Síndrome de Eagle, além de abordar os métodos de diagnóstico e estratégias terapêuticas. Materiais e métodos: Foi realizada uma busca por artigos científicos publicados no período de 2016 a 2024, utilizando as bases de dados Scientific Electronic Library Online (SciELO), US National Library of Medicine (PubMed) e Google Scholar. A coleta de artigos foi realizada nos idiomas inglês e português, utilizando as palavras-chave: "síndrome de eagle", "síndrome estiloide", "síndrome da artéria carótida", "estilalgia", "eagle syndrome", "styloid syndrome", "carotid artery syndrome" e "stylalgia". Conclusão: Os profissionais devem estar atentos à síndrome de Eagle em casos de dor unilateral ao realizar atividades como engolir, bocejar e chorar, sem causa aparente, especialmente em mulheres adultas que não encontram alívio com analgésicos. Devido à frequência de casos assintomáticos, a realização precoce de exames radiológicos desempenha um papel crucial na avaliação diagnóstica. É essencial que profissionais de Otorrinolaringologia, Neurologia e Odontologia estejam cientes dessa síndrome, pois está associada a uma significativa deterioração na qualidade de vida. (AU)

Objective: This work aims to provide a comprehensive analysis of the anatomical, clinical and radiographic characteristics of Eagle Syndrome, in addition to addressing diagnostic methods and therapeutic strategies. Materials and methods: A search was carried out for scientific articles published between 2016 and 2024, using the Scientific Electronic Library Online (SciELO), US National Library of Medicine (PubMed) and Google Scholar databases. Articles were collected in English and Portuguese, using the keywords: "eagle syndrome", "styloid syndrome", "carotid artery syndrome", "stilalgia", "eagle syndrome", "styloid syndrome", "carotid artery syndrome" and "stylalgia". Conclusion: Professionals should be aware of Eagle syndrome in cases of unilateral pain when performing activities such as swallowing, yawning and crying, without an apparent cause, especially in adult women who do not find relief with analgesics. Due to the frequency of asymptomatic cases, early radiological examinations play a crucial role in diagnostic evaluation. It is essential that Otorhinolaryngology, Neurology and Dentistry professionals are aware of this syndrome, as it is associated with a significant deterioration in quality of life. (AU)

Eagle's syndrome: a piercing matter.

We present an unusual case of Eagle's syndrome with bilateral internal carotid artery dissection in a 45-year-old man. Initial symptomatology included ipsilateral headaches and facial sensory symptoms. A right horner's syndrome was present on clinical examination. Radiological imaging revealed an old infarct, with bilateral carotid dissections and bilateral elongated styloid processes consistent with Eagle's syndrome. Despite initiation of secondary prevention with antiplatelet therapy, he had two further ischaemic events. The case highlights the symptomatology and complications of Eagle's syndrome, with its management discussed through a review of similar case reports.

Repair of Tegmen Tympani Defect Presenting with Spontaneous Cerebrospinal Fluid Otorrhea Using the Middle Cranial Fossa Approach.

Spontaneous cerebrospinal fluid (CSF) otorrhea frequently occurs without temporal bone trauma, fracture, surgery, or any identifiable causes. It is usually associated with tegmen tympani defects of the temporal bone in adults. The congenital origin theory and the arachnoid granulation theory have been accepted to explain the tegmen tympani defect. Magnetic resonance imaging (MRI) and computed tomography (CT) are used to show the defects, brain tissue, and the meninges. We recently encountered three cases of spontaneous CSF otorrhea with a defect on the tegmental plate of the temporal bone. High-resolution CT (HRCT) scan of the temporal bones showed the tegmen tympani defects. The defects were successfully repaired with temporal muscle fascia or fascia lata graft and fibrin glue using the middle cranial fossa approach via craniotomy.

Use of an intraoperative navigation system and piezoelectric surgery for styloidectomy in a patient with Eagle's syndrome: a case report.

BACKGROUND: Elongated styloid process syndrome (Eagle's syndrome) is the term given to the symptomatic elongation of the styloid process or the mineralization of the stylohyoid or stylomandibular ligament. The two commonly used approaches for the surgical treatment of this syndrome are the transcervical and transoral approaches. Both have their limitations and specific intraoperative risks. Here, we report the treatment of a patient with Eagle's syndrome using the transoral approach in conjunction with piezoelectric surgery, surgical planning, and intraoperative navigation to reduce the risk of complications. CASE PRESENTATION: The elongated styloid process was resected in a 45-year-old Japanese man using a minimally invasive approach with an intraoperative navigation system. Preoperative preparation involved the use of a custom interocclusal splint to produce the mouth opening conditions required during surgery. Using the three-dimensional position of the navigation probe, the location of the elongated styloid process was identified. After confirmation of the resection spot via the transoral approach, the styloid process was dissected by piezoelectric surgery. Follow-up examination showed an uneventful recovery with no associated complications. CONCLUSION: The resection of the styloid process using an intraoperative navigation system and a custom interocclusal splint during a transoral approach, together with a piezoelectric cutting device, is safe and effective for the treatment of Eagle's syndrome.

[Application of nasal endoscopy in styloid process truncating operation].

OBJECTIVE: To investigate the application and the clinical effect of nasal endoscope in styloid process truncating operation. METHOD: Dividing 66 patients into nasal endoscope group and control group randomly, and then observe them. Nasal endoscope group were cured with nasal endoscope during the styloid process truncating operation and the control group were treated with the routine method of intraoral path. All the operations were executed by the same person. Observation index included the following aspects: the length of the truncated styloid process, the operation time, intraoperative bleeding, postoperative pain and pain degree, inflammatory reaction of raw surface and rehaemorrhagia. RESULT: Compared with the control group, the length of the truncated styloid process of nasal endoscope group were longer and the operation time had no significant differences. The intraoperative bleeding of intraoperative bleeding group were (85.0 +/- 35.0)ml and in control group were (95.0 +/- 40.0)ml. There are no significant differences between the result. Nasal endoscope group also had the advantages of fewer aches and less inflammatory reaction of raw surface. Both of the team had no rehaemorrhagia. CONCLUSION: nasal endoscope can truncate a longer styloid process in styloid process truncating operation and is effective to relieve pain, and ease inflammatory reaction, it's a safe mini-trauma operation.

Carotid artery dissection due to elongated styloid process: a self-stabbing phenomenon.

Elongated styloid process (ESP) is an anatomical variant that has been described as the cause of Eagle syndrome. Until recently, the styloid process has not been appreciated as a significant contributor to carotid artery dissection (CAD), which is not part of Eagle syndrome. We present a case of a 41-year-old male who presented with acute right middle cerebral artery occlusion and was found to have ESP projecting to and abutting the lateral wall of a dissected right internal carotid artery (ICA). Forced sustained head turning with maximal muscle contraction was the initiating event driving the styloid process into the wall of the ICA in a manner that can be likened to being stabbed with a pointed object. Knowing the association between ESP, Eagle syndrome, and CAD shall lead to increased awareness and appropriate diagnosis and treatment.

Eagle's syndrome, a rare cause of neck pain.

Eagle's syndrome (ES) sometimes called styloid or stylohyoid syndrome is defined as the symptomatic elongation of the styloid process or mineralisation of the stylohyoid ligament. The symptoms related to this condition can be confused with those attributed to a wide variety of facial neuralgias. We report a 34-year-old male patient who presented with a recurrent neck pain and dysphagia. He was diagnosed to have an elongated styloid process. An intraoral transtonsillar shortening of the styloid process was done. We discuss the clinical presentation, diagnosis and treatment of ES as well as a review of the literature.