2025 年 19 巻 1 号 論文ID: cr.2025-0111
Objective: Cerebral venous sinus thrombosis (CVST) is a rare but serious condition that can cause intracranial hypertension and hemorrhage. Common risk factors include thrombophilia and oral contraceptive use, but jugular Eagle syndrome—caused by an elongated styloid process compressing the internal jugular vein—is an uncommon yet clinically significant etiology.
Case Presentation: A 44-year-old woman presented with severe right-sided headache, neck pain, and visual disturbances after cervical spine manipulation. Imaging revealed extensive CVST with venous congestion hemorrhage, and jugular Eagle syndrome was confirmed. Symptoms persisted despite anticoagulation, but her headache improved after successful endovascular thrombectomy. Styloidectomy was considered for long-term management.
Conclusion: Jugular Eagle syndrome is a rare cause of CVST. Anticoagulation remains first-line, but thrombectomy may be required in refractory cases, and surgical styloidectomy should be considered for definitive management.
Cerebral venous sinus thrombosis (CVST) is an uncommon but potentially life-threatening cerebrovascular disorder that can result in intracranial hypertension, venous congestion, and secondary hemorrhage.1) The condition is typically associated with risk factors such as thrombophilia, oral contraceptive use, malignancy, and systemic inflammatory diseases.2)
Eagle syndrome is a rare disorder caused by elongation of the styloid process, which may lead to cranial nerve irritation or vascular compression.3) Its vascular subtype, known as jugular Eagle syndrome, occurs when the elongated styloid process compresses the internal jugular vein, thereby impairing cerebral venous outflow. This can result in intracranial hypertension and, in rare instances, CVST.3) Jugular Eagle syndrome is increasingly recognized as an underdiagnosed cause of chronic cerebral venous hypertension, with clinical manifestations ranging from headache and visual disturbances to cognitive dysfunction and stroke-like events.4)
Management of CVST primarily relies on systemic anticoagulation, which prevents thrombus propagation and facilitates recanalization. However, in patients with severe venous congestion or clinical deterioration despite anticoagulation, endovascular thrombectomy may provide rapid symptom relief and restoration of venous flow.3,4) For patients with persistent venous obstruction due to bony compression, surgical resection of the elongated styloid process (styloidectomy) represents a definitive therapeutic option.2)
Here, we present a rare case of CVST secondary to jugular Eagle syndrome, successfully treated with endovascular thrombectomy, with styloidectomy considered for long-term management. This case underscores the importance of recognizing mechanical venous outflow obstruction as a potential etiology of CVST and highlights the role of a multidisciplinary approach in optimizing patient outcomes.
A 44-year-old woman presented to the emergency department with severe right-sided headache and neck pain. Symptoms had begun 1 week earlier after a bicycle accident followed by cervical spine manipulation. She also reported intermittent visual disturbances described as “flashes” and a single episode of vomiting.
On neurological examination, the patient was alert and fully oriented, with no focal deficits. However, there was marked tenderness along the right side of the neck. No signs of systemic infection or other illness were identified.
Imaging and diagnosisCTA demonstrated extensive thrombosis involving the superior sagittal sinus, right transverse sinus, and sigmoid sinus, extending into the right internal jugular vein. Secondary findings included a venous congestion hemorrhage in the right temporal lobe and a small subarachnoid hemorrhage (Fig. 1).
Subsequent imaging revealed an elongated right styloid process compressing the internal jugular vein, consistent with jugular Eagle syndrome (Fig. 2). Extended coagulation studies excluded an underlying thrombophilic disorder.
Therapeutic anticoagulation with intravenous heparin was initiated and later transitioned to oral apixaban (5 mg twice daily). Despite treatment, the patient developed progressive headache and clinical deterioration. On the same day, endovascular thrombectomy was performed. Thrombectomy was chosen because of acute clinical deterioration and the extensive involvement of multiple venous sinuses despite adequate therapeutic anticoagulation. Local catheter-directed thrombolysis was also considered; however, given the presence of venous congestion hemorrhage, the risk of exacerbating intracranial bleeding was considered too high. Therefore, mechanical thrombectomy with stent retriever and aspiration techniques was selected as the safest and most effective option in this setting.
A transvenous approach using a stent retriever in combination with aspiration achieved near-complete recanalization of the affected venous sinuses (Fig. 3). Post-procedurally, her neurological status remained stable. Headaches initially persisted but improved significantly over several days, permitting gradual tapering of analgesics.
The patient was discharged in stable condition with mild, well-controlled residual headaches. Given the ongoing jugular venous compression, surgical styloidectomy was discussed as a definitive strategy for long-term management.
At the 3-month follow-up, the patient remained clinically stable without recurrence of thrombosis. Headaches were mild and well-controlled, and further surgical evaluation for styloidectomy was ongoing.
This case highlights an uncommon but clinically significant cause of CVST: mechanical compression of the internal jugular vein due to an elongated styloid process, also termed jugular Eagle syndrome. The principal finding is that venous outflow obstruction from this rare anatomical abnormality can precipitate acute CVST, and that endovascular thrombectomy may be necessary when anticoagulation alone is insufficient. Furthermore, long-term management requires consideration of surgical styloidectomy to address the underlying mechanical obstruction and prevent recurrence.
Jugular Eagle syndrome as a cause of CVSTCVST is an uncommon but potentially devastating cerebrovascular condition, accounting for less than 1% of all strokes.5) It is typically associated with prothrombotic conditions such as inherited thrombophilia, malignancy, infection, trauma, and hormonal risk factors such as oral contraceptive use.2,6) Mechanical venous outflow obstruction as a precipitating cause remains poorly recognized.
Eagle syndrome, first described by Watt Eagle in 1937, traditionally refers to a symptomatic elongation of the styloid process leading to craniofacial pain, dysphagia, or cranial nerve irritation.1,3,7) The true incidence of jugular vein stenosis due to Eagle syndrome remains uncertain. Systematic reviews indicate that only small series and isolated case reports are available, suggesting that the condition is rare but likely underdiagnosed.4–8) With the growing use of advanced venous imaging, jugular Eagle syndrome is being recognized with increasing frequency in patients presenting with unexplained intracranial hypertension or venous congestion. The vascular subtype, jugular Eagle syndrome, occurs when the styloid process impinges on the internal jugular vein, resulting in venous outflow obstruction. Although most reported patients present with headache, pulsatile tinnitus, or features of idiopathic intracranial hypertension, this condition has only rarely been associated with CVST.4,7)
In the present case, the elongated styloid process produced significant narrowing of the right internal jugular vein between the styloid process and the transverse process of C2. Bilateral elongation of the styloid processes was present, and both internal jugular veins were narrowed. However, the right-sided compression was more severe and was likely the dominant contributor to venous outflow obstruction and thrombosis. This anatomical relationship has been described in several case reports and small series,5,6) and is increasingly recognized as a predisposing factor for venous hypertension and thrombosis. Chronic jugular compression may force collateral venous drainage through less efficient pathways, raising intracranial pressure and reducing cerebral perfusion.5) Our patient’s extensive thrombosis involving multiple sinuses underscores the potential for serious complications when collateral circulation is insufficient.
Increasing attention has also been given to jugular vein compression syndromes beyond CVST.5,6) Several studies have highlighted their overlap with idiopathic intracranial hypertension, in which impaired venous outflow may contribute to chronically elevated intracranial pressure and headache symptoms.4–8) This suggests that jugular Eagle syndrome may share pathophysiological mechanisms with other venous outflow disorders and underscores the importance of careful venous imaging in patients presenting with unexplained intracranial hypertension.
Comparison with existing literatureThe available literature on jugular Eagle syndrome and CVST is sparse, consisting mainly of case reports and small series.2,4,7,8) Zhang et al. reported a patient with jugular Eagle syndrome–related CVST who declined surgery and was managed conservatively with anticoagulation.2) In contrast, Werheim et al. described successful symptom resolution following styloidectomy in a similar case.4) Other observational studies suggest that patients with jugular vein compression may present with diverse neurological phenotypes, ranging from headache and visual disturbance to cognitive impairment and transient ischemic episodes.2,8,9)
To our knowledge, only a few cases of CVST associated with jugular Eagle syndrome have been reported in the literature.1,4,8,9) Unlike previous cases that were managed conservatively with anticoagulation or surgery alone, our patient experienced acute clinical deterioration despite anticoagulation and required escalation to mechanical thrombectomy. This highlights the potential role of thrombectomy as an adjunctive therapy in severe cases with rapid progression.
Our case contributes to this literature by illustrating the role of thrombectomy as an adjunct to anticoagulation in the acute setting. Although anticoagulation remains the cornerstone of CVST management, some patients experience clinical deterioration or fail to improve. Several multicentre experiences and systematic reviews support the use of mechanical thrombectomy in carefully selected patients with severe venous congestion, where rapid recanalization can reduce intracranial pressure and relieve refractory symptoms.9,10) In our patient, stent retriever and aspiration techniques achieved near-complete recanalization with progressive symptom improvement, which parallels the outcomes reported in other modern series.10)
Clinical implicationsThe management of CVST in the setting of jugular Eagle syndrome presents unique challenges. Anticoagulation prevents thrombus propagation and promotes recanalization, but does not address the underlying mechanical obstruction. Thrombectomy can rapidly restore venous flow, but carries procedural risks including vessel injury and hemorrhage, and its long-term benefit in preventing recurrence remains uncertain.11,12)
The definitive management of jugular Eagle syndrome with associated CVST requires correction of the anatomical cause. Styloidectomy, performed via either an intraoral or external approach, has been reported to relieve jugular venous compression and improve symptoms in patients with Eagle syndrome.3,5,8) Held et al. reported favorable outcomes in a large series of 56 patients, most of whom underwent intraoral surgery with minimal complications.5) A systematic review by Badhey et al. similarly concluded that surgical removal of the elongated styloid process is safe and effective, particularly in refractory cases.3) In our case, styloidectomy was initially deferred in the acute phase due to the severity of thrombosis and the priority of re-establishing venous patency, but remains a strong consideration for long-term prevention of recurrence.
More explicitly, styloidectomy was postponed because surgery in the acute phase carried a significant risk in the setting of active cerebral venous thrombosis and associated intracranial hemorrhage. The immediate clinical priority was to stabilize the patient and restore venous outflow. The patient has since been referred for surgical evaluation, and styloidectomy remains under consideration as a definitive strategy for long-term management and prevention of recurrence.
This case underscores the importance of maintaining a broad differential diagnosis when evaluating patients with CVST, especially in the absence of conventional risk factors. Imaging of the jugular veins and bony anatomy should be considered in patients with unexplained CVST or features suggestive of venous outflow obstruction. Increased awareness among neurologists, radiologists, and head and neck surgeons is essential to avoid diagnostic delays.
LimitationsOur findings are limited by the single-patient design, which restricts generalizability. The causal relationship between jugular compression and CVST cannot be definitively proven, although the close anatomical correlation, absence of other risk factors, and clinical improvement following recanalization strongly support the association. Long-term follow-up is also needed to assess the durability of symptom control and the role of styloidectomy in preventing recurrence. In our patient, follow-up at 3 months demonstrated stable neurological status, no recurrence of thrombosis, and only mild residual headache, while surgical planning for styloidectomy is in progress.
Larger studies and registries are warranted to clarify patient selection criteria and to guide evidence-based management.
This case illustrates that jugular Eagle syndrome is a rare but important cause of CVST. Clinicians should consider mechanical venous outflow obstruction when evaluating patients with unexplained venous thrombosis or intracranial hypertension. Anticoagulation remains first-line treatment, but thrombectomy may provide life-saving benefit in cases of severe venous congestion or refractory symptoms. Ultimately, surgical styloidectomy should be considered as a definitive therapy for patients with persistent venous outflow obstruction.
A multidisciplinary approach involving neurologists, interventional neuroradiologists, and head and neck surgeons is crucial for optimizing outcomes. Increasing clinical awareness and reporting of similar cases will help to build the evidence base for diagnosis and management of this under-recognized condition.
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Ethical approvalThis case report did not require formal review by an ethics committee, as it is a retrospective analysis of a single patient and does not involve experimental procedures. In addition, the present case report is not a first-in-human report of a novel technology or surgical technique; therefore, a registration of this case report under the Declaration of Helsinki 2013 is not required.
Consent for publicationWritten informed consent was obtained from the patient on January 25, 2025, for the publication of this case report and accompanying images.
Disclosure statementThe authors have no conflicts of interest to declare.
