Yoshii Toshitaka, Hirai Takashi, Egawa Satoru, Hashimoto Motonori, Matsukura Yu, Inose Hiroyuki, Sanjo Nobuo, Yokota Takanori, Okawa Atsushi
Department of Orthopaedic and Spinal Surgery, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.
Department of Neurology, Tokyo Medical and Dental University, Tokyo, Japan.
Front Neurol. 2022 Jul 13;13:919280. doi: 10.3389/fneur.2022.919280. eCollection 2022.
Superficial siderosis (SS) of the central nervous system is a rare disease caused by chronic and repeated hemorrhages in the subarachnoid space. Recently, attention has been paid on the association of SS and dural defect with ventral fluid-filled collection in the spinal canal (VFCC). The pathophysiology of hemosiderin deposition in patients with SS and dural defects is still unclear. However, previous studies have suggested the possible mechanism: cerebrospinal fluid (CSF) leaks into the epidural space through the ventral dural defect, and repetitive bleeding occurs from the epidural vessels that circulate back to the subarachnoid space through the dural defect, leading to hemosiderin deposition on the surface of the brain, the central nerves, and the spinal cord. Previously, the surgical closure of dural defect the posterior approach has been reported to be effective in arresting the continued subarachnoid bleeding and disease progression. Herein, we describe SS cases whose dural defects were repaired the anterior approach. From the direct anterior approach to the ventral dural defect findings, we confirmed that the outer fibrous dura layer is intact, and the defect is localized in the inner thin layer. From the findings of this study, our proposed theory is that dural tear at the inner dural layer causes "dural dissection," which is likely to occur between the outer fibrous layer and inner dural border cellular layer. Bleeding from the vessels between the inner and outer Line 39-40 dural layers seems to be the pathology of SS with dural defect.
中枢神经系统表面铁沉积症(SS)是一种由蛛网膜下腔慢性反复出血引起的罕见疾病。最近,SS与硬脑膜缺损以及椎管内腹侧液性聚集(VFCC)之间的关联受到了关注。SS合并硬脑膜缺损患者中含铁血黄素沉积的病理生理学仍不清楚。然而,先前的研究提出了可能的机制:脑脊液(CSF)通过腹侧硬脑膜缺损漏入硬膜外间隙,硬膜外血管反复出血,这些血液通过硬脑膜缺损回流至蛛网膜下腔,导致含铁血黄素在脑表面、中枢神经和脊髓表面沉积。此前,据报道采用后路手术封闭硬脑膜缺损对阻止持续的蛛网膜下腔出血和疾病进展有效。在此,我们描述了采用前路手术修复硬脑膜缺损的SS病例。从直接前路观察腹侧硬脑膜缺损的情况,我们证实外层纤维性硬脑膜层完整,缺损位于内层薄层。根据本研究的结果,我们提出的理论是,内层硬脑膜层的硬脑膜撕裂导致“硬脑膜剥离”,这种情况很可能发生在外层纤维层与内层硬脑膜边界细胞层之间。内层和外层硬脑膜层之间血管的出血似乎是合并硬脑膜缺损的SS的病理表现。