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[急性脊髓病的一种罕见病因:颅颈交界区硬脊膜动静脉瘘]

[An unusual cause of acute myelopathy: a dural arteriovenous fistula at the craniocervical junction].

作者信息

Slaba S, Smayra T, Hage P, Okais N, Atallah N

机构信息

Service de Radiodiagnostic, Hôtel-Dieu de France (HDF), Beyrouth, Liban.

出版信息

J Med Liban. 2000 May-Jun;48(3):168-72.

PMID:11268573
Abstract

BACKGROUND

Dural arteriovenous fistulas (DAVF) account for 10% to 15% of all intracranial arteriovenous malformations. Since the first case published by Woimant et al. in 1982, many type V DAVF, i.e. with spinal venous drainage, have been reported. Fistulas located at the craniocervical junction (CCJ) however, are exceptional and only 10 cases of CCJ fistulas associated with myelopathy have been described.

CASE REPORT

The authors present a 36-year-old male patient without previous medical history, suffering from acute myelopathy. Cervical MRI showed multiple serpiginous flow-voids along the cord surface and cerebral angiography disclosed a dural fistula of the CCJ fed by the right posterior meningeal and occipital arteries. The venous drainage was directed caudally towards the perimedullary veins. Embolization through the occipital artery, using cyanoacrylate, was performed and resulted in complete cure of the malformation with rapid clinical recovery.

DISCUSSION

The authors discuss the pathophysiology and clinical consequences of intracranial DAVF with myelopathy (named V, m+), that are usually identical to those of spinal dural fistulas and related to intramedullary venous hypertension. Early treatment is essential to reverse the patient's myelopathy. Embolization, if technically possible, is the preferred treatment and cyanoacrylate remains the best embolic agent. Following glue deposition, systemic high-dose steroids should be administered to prevent edema.

CONCLUSION

In conclusion, this is the first case of DAVF of the foramen magnum causing myelopathy to be detected early and cured by glue embolization alone, with rapid and total clinical recovery.

摘要

背景

硬脑膜动静脉瘘(DAVF)占所有颅内动静脉畸形的10%至15%。自1982年沃伊曼特等人发表首例病例以来,已报道了许多V型DAVF,即伴有脊髓静脉引流的病例。然而,位于颅颈交界区(CCJ)的瘘极为罕见,仅有10例与脊髓病相关的CCJ瘘被描述。

病例报告

作者介绍了一名36岁无前科病史的男性患者,患有急性脊髓病。颈椎MRI显示脊髓表面有多个蜿蜒的流空影,脑血管造影显示CCJ处有一个由右后脑膜动脉和枕动脉供血的硬脑膜瘘。静脉血引流向尾侧至脊髓周围静脉。采用氰基丙烯酸酯通过枕动脉进行栓塞,畸形完全治愈,临床迅速恢复。

讨论

作者讨论了伴有脊髓病的颅内DAVF(命名为V,m+)的病理生理学和临床后果,其通常与脊髓硬脑膜瘘相同,与髓内静脉高压有关。早期治疗对于逆转患者的脊髓病至关重要。如果技术上可行,栓塞是首选治疗方法,氰基丙烯酸酯仍然是最佳栓塞剂。注入胶水后,应给予全身大剂量类固醇以预防水肿。

结论

总之,这是首例枕骨大孔区DAVF导致脊髓病且早期被发现并仅通过胶水栓塞治愈、临床迅速完全恢复的病例。

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引用本文的文献

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Front Neurol. 2023 Apr 18;14:1121075. doi: 10.3389/fneur.2023.1121075. eCollection 2023.
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Arteriovenous fistulas at the cervicomedullary junction presenting with subarachnoid hemorrhage: six case reports with special reference to the angiographic pattern of venous drainage.颈髓交界处动静脉瘘伴蛛网膜下腔出血:6例报告并特别提及静脉引流的血管造影模式
AJNR Am J Neuroradiol. 2005 Sep;26(8):1949-54.