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可能因先天性神经胶质间充质异常导致马让迪孔阻塞相关的脊髓空洞症

Syringomyelia Associated with Magendie's Foramen Obstruction Due to Probably Congenital Gliomesenchymal Anomaly.

作者信息

Kumagai Shunsuke, Ogura Ryosuke, Shibuya Kohei, On Jotaro, Saito Shoji, Sano Masakazu, Kakita Akiyoshi, Oishi Makoto

机构信息

Department of Neurosurgery, Brain Research Institute, Niigata University, Niigata, Niigata, Japan.

Department of Pathology, Brain Research Institute, Niigata University, Niigata, Niigata, Japan.

出版信息

NMC Case Rep J. 2025 Jun 4;12:221-226. doi: 10.2176/jns-nmc.2025-0041. eCollection 2025.

Abstract

We report a case of syringomyelia in a 55-year-old man with a unique obstruction of Magendie's foramen. Spinal magnetic resonance imaging revealed a large syrinx extending from C1 to C3, with intermittent syringomyelia extending down to Th11. While the obstruction was not clearly evident on T2-weighted imaging, three-dimensional constructive interference in steady-state imaging demonstrated a thickened membranous tissue blocking the cerebrospinal fluid outlet, leading to syrinx formation. Surgical intervention, involving the resection of the thickened membrane to open the foramen of Magendie, resulted in considerable improvement in the syringomyelia and neurological symptoms. Histopathological examination revealed gliomesenchymal tissue, suggesting an embryonic origin of the obstruction. This case highlights the importance of detailed imaging, particularly three-dimensional constructive interference in steady-state sequence, in diagnosing foramen of Magendie obstruction and the potential for successful surgical treatment in selected cases. Histopathological examination is crucial for differentiating gliomesenchymal tissue from adhesive arachnoiditis.

摘要

我们报告一例55岁男性的脊髓空洞症,其存在独特的马让迪孔梗阻。脊髓磁共振成像显示一个从C1延伸至C3的大空洞,间歇性脊髓空洞症向下延伸至T11。虽然在T2加权成像上梗阻并不明显,但稳态三维建设性干扰成像显示有增厚的膜状组织阻塞脑脊液出口,导致空洞形成。手术干预包括切除增厚的膜以打开马让迪孔,使脊髓空洞症和神经症状有了显著改善。组织病理学检查显示为胶质间充质组织,提示梗阻起源于胚胎。该病例强调了详细成像的重要性,尤其是稳态三维建设性干扰序列成像,对于诊断马让迪孔梗阻以及在特定病例中成功进行手术治疗的可能性。组织病理学检查对于区分胶质间充质组织和粘连性蛛网膜炎至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fffc/12187467/c2ad7751042e/2188-4226-12-0221-g001.jpg

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