Department of Neurosurgery, Osaka Police Hospital, 10-31 Kitayama-Cho, Tennoji-Ku, Osaka City, 543-0035, Japan.
Acta Neurochir (Wien). 2024 Jan 17;166(1):20. doi: 10.1007/s00701-024-05900-x.
Eagle jugular syndrome (EJS), recently identified as a cause of cerebrovascular disease (CVD) due to venous obstruction by an elongated styloid process (SP), is reported here alongside a case of concurrent de novo cerebral cavernous malformation (CCM). This study aims to explore the potential causal relationship between EJS and de novo CCM through a comprehensive literature review.
Systematic literature reviews, spanning from 1995 to 2023, focused on EJS cases with definitive signs and symptoms and de novo CCM cases with detailed clinical characteristics. Data on the pathophysiology and clinical manifestations of EJS, as well as potential risk factors preceding de novo CCM, were collected to assess the relationship between the two conditions.
Among 14 patients from 11 articles on EJS, the most common presentation was increased intracranial hypertension (IIH), observed in 10 patients (71.4%), followed by dural sinus thrombosis in four patients (28.6%). In contrast, 30 patients from 28 articles were identified with de novo CCM, involving 37 lesions. In these cases, 13 patients developed CCM subsequent to developmental venous anomalies (43%), seven following dural arteriovenous fistula (dAVF) (23%), and two after sinus thrombosis (6%). In a specific case of de novo brainstem CCM, the development of an enlarged condylar emissary vein, indicative of venous congestion due to IJV compression by the elongated SP, was noted before the emergence of CCM.
This study underscores that venous congestion, a primary result of symptomatic EJS, might lead to the development of de novo CCM. Thus, EJS could potentially be an indicator of CCM development. Further epidemiological and pathophysiological investigations focusing on venous circulation are necessary to clarify the causal relationship between EJS and CCM.
鹰颈静脉综合征（Eagle jugular syndrome，EJS）最近被认为是由于茎突过长导致静脉阻塞而引起的脑血管疾病（Cerebrovascular disease，CVD）的病因之一。本文报告了一例同时发生的新发性脑动静脉畸形（Cerebral cavernous malformation，CCM）病例。本研究旨在通过全面的文献回顾，探讨 EJS 与新发性 CCM 之间的潜在因果关系。
系统文献综述，时间跨度为 1995 年至 2023 年，重点关注有明确症状和体征的 EJS 病例，以及有详细临床特征的新发性 CCM 病例。收集有关 EJS 的病理生理学和临床表现以及新发性 CCM 之前潜在风险因素的数据，以评估这两种疾病之间的关系。
在 11 篇关于 EJS 的文章中，有 14 例患者，最常见的表现是颅内压增高（10 例，71.4%），其次是 4 例硬脑膜窦血栓形成（28.6%）。相比之下，在 28 篇文章中有 30 例患者患有新发性 CCM，涉及 37 个病变。在这些病例中，13 例 CCM 继发于发育性静脉异常（43%），7 例继发于硬脑膜动静脉瘘（dural arteriovenous fistula，dAVF）（23%），2 例继发于窦血栓形成（6%）。在一例新发性脑干部位 CCM 病例中，发现一个扩大的髁导静脉，这表明由于颈内静脉受压，静脉淤血，导致 CCM 出现。
本研究强调了症状性 EJS 引起的静脉淤血可能导致新发性 CCM 的发生。因此，EJS 可能是 CCM 发生的一个指标。需要进一步进行有关静脉循环的流行病学和病理生理学研究，以阐明 EJS 与 CCM 之间的因果关系。
