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海绵状血管畸形:自然病史、诊断与治疗。

Cavernous malformations: natural history, diagnosis and treatment.

机构信息

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.

出版信息

Nat Rev Neurol. 2009 Dec;5(12):659-70. doi: 10.1038/nrneurol.2009.177.

DOI:10.1038/nrneurol.2009.177
PMID:19953116
Abstract

Cavernous malformations (CMs) consist of dilated vascular channels that have a characteristic appearance on MRI. CMs are usually found intracranially, although such lesions can also affect the spinal cord. Individuals with CMs can present with epilepsy and focal neurological deficits or acute intracranial hemorrhage. In many cases, however, patients with such lesions are asymptomatic at diagnosis. Furthermore, several natural history studies have documented that a substantial proportion of asymptomatic CMs follow a benign course. Surgical resection is recommended for CMs that require intervention. Radiosurgery has been advocated for many lesions that have not been easily accessible by conventional surgery. The outcomes of radiosurgery and surgery for deep lesions, however, vary widely between studies, rendering treatment recommendations for such CMs difficult to make. In addition to reviewing the literature, this article will discuss the current understanding of lesion pathophysiology and explore the controversial issues in the management of CMs, such as when to use radiosurgery or surgery in deep-seated lesions, the treatment of epilepsy, and the safety of anticoagulation.

摘要

海绵状血管畸形(CM)由扩张的血管通道组成,在 MRI 上具有特征性表现。CM 通常在颅内发现,尽管此类病变也可能影响脊髓。CM 患者可出现癫痫和局灶性神经功能缺损或急性颅内出血。然而,在许多情况下,此类病变患者在诊断时无症状。此外,几项自然病史研究表明,相当一部分无症状的 CM 呈良性病程。需要干预的 CM 推荐手术切除。放射外科已被用于许多传统手术难以触及的病变。然而,放射外科和手术治疗深部病变的结果在不同的研究中差异很大,使得难以对这些 CM 做出治疗建议。除了回顾文献外,本文还将讨论目前对病变病理生理学的理解,并探讨 CM 管理中的争议问题,例如何时在深部病变中使用放射外科或手术、癫痫的治疗以及抗凝的安全性。

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本文引用的文献

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Follow-up MR of Intracranial Cavernomas The Relationship between Haemorrhagic Events and Morphology.颅内海绵状血管瘤的随访磁共振成像:出血事件与形态学之间的关系
Interv Neuroradiol. 1996 Jun 30;2(2):127-35. doi: 10.1177/159101999600200205. Epub 2001 May 15.
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Seizure characteristics and control after microsurgical resection of supratentorial cerebral cavernous malformations.幕上脑海绵状血管畸形显微手术后的癫痫发作特征及控制情况
Neurosurgery. 2009 Jul;65(1):31-7; discussion 37-8. doi: 10.1227/01.NEU.0000346648.03272.07.
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Radiosurgery for angiographically occult vascular malformations.
西班牙裔老年海绵状脑畸形患者的代谢综合征与出血性中风
Diagnostics (Basel). 2025 Apr 30;15(9):1144. doi: 10.3390/diagnostics15091144.
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Giant pediatric brainstem cavernoma: illustrative case.巨大型小儿脑干海绵状血管瘤:病例说明
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Cavernous malformations of the optic nerve and chiasm: illustrative case.视神经和视交叉海绵状畸形:病例说明
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Clinical, genomic, and histopathologic diversity in cerebral cavernous malformations.脑海绵状血管畸形的临床、基因组和组织病理学多样性。
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Cerebral vascular malformations: pathogenesis and therapy.脑血管畸形:发病机制与治疗
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Advanced Diffusion MRI in the Differential Diagnosis of Hemorrhagic Cavernous Malformations in the Brain: A Case Report.高级扩散磁共振成像在脑内出血性海绵状畸形鉴别诊断中的应用:一例报告
Cureus. 2024 Sep 10;16(9):e69095. doi: 10.7759/cureus.69095. eCollection 2024 Sep.
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Long-Term Outcomes of Gamma Knife Radiosurgery for Cerebral Cavernous Malformations: 10 Years and Beyond.伽玛刀放射外科治疗脑海绵状血管畸形的长期结果:10 年及以上。
J Korean Med Sci. 2024 Aug 19;39(32):e229. doi: 10.3346/jkms.2024.39.e229.
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Multiple Cavernous Malformation Syndrome in an Infant: A Case Report.婴儿多发性海绵状血管畸形综合征:一例报告
Cureus. 2024 Jul 3;16(7):e63750. doi: 10.7759/cureus.63750. eCollection 2024 Jul.
血管造影隐匿性血管畸形的放射外科治疗。
Neurosurg Focus. 2009 May;26(5):E16. doi: 10.3171/2009.2.FOCUS0923.
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Brainstem cavernous malformations.脑干海绵状血管畸形
Neurosurgery. 2009 May;64(5):E805-18; discussion E818. doi: 10.1227/01.NEU.0000343668.44288.18.
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Immune response in human cerebral cavernous malformations.人脑海绵状血管畸形中的免疫反应。
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The cerebral cavernous malformation signaling pathway promotes vascular integrity via Rho GTPases.脑海绵状血管畸形信号通路通过Rho GTP酶促进血管完整性。
Nat Med. 2009 Feb;15(2):177-84. doi: 10.1038/nm.1911. Epub 2009 Jan 18.
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Regulation of cardiovascular development and integrity by the heart of glass-cerebral cavernous malformation protein pathway.玻璃脑海绵状血管畸形蛋白通路的心脏对心血管发育和完整性的调节
Nat Med. 2009 Feb;15(2):169-76. doi: 10.1038/nm.1918. Epub 2009 Jan 18.
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Exp Cell Res. 2009 Jan 15;315(2):285-303. doi: 10.1016/j.yexcr.2008.10.006. Epub 2008 Oct 21.
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Hemorrhage from cavernous malformations of the brain: definition and reporting standards. Angioma Alliance Scientific Advisory Board.脑海绵状血管畸形出血:定义与报告标准。血管瘤联盟科学咨询委员会。
Stroke. 2008 Dec;39(12):3222-30. doi: 10.1161/STROKEAHA.108.515544. Epub 2008 Oct 30.