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脊髓髓内海绵状血管瘤:临床表现、病理特征及手术治疗

Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management.

作者信息

Ogilvy C S, Louis D N, Ojemann R G

机构信息

Neurosurgical Service, Massachusetts General Hospital, Boston.

出版信息

Neurosurgery. 1992 Aug;31(2):219-29; discussion 229-30. doi: 10.1227/00006123-199208000-00007.

Abstract

Cavernous angiomas of the spinal cord are rare lesions that can cause severe neurological symptoms. We add a series of 6 patients with intramedullary cavernous angiomas of the spinal cord to 30 patients with 31 histologically verified lesions already described in the literature. Four types of clinical presentation were seen in the 36 patients: 1) discrete episodes of neurological deterioration with varying degrees of recovery between episodes (13 patients); 2) slow progression of neurological decline (12 patients); 3) acute onset of symptoms with rapid decline (8 patients); and 4) acute onset of mild symptoms with subsequent gradual decline lasting weeks to months (3 patients). Of the 36 patients, 25 (69%) were women and 11 (31%) were men. The peak age of presentation was in the fourth decade. The thoracic spinal cord was affected in more than half the patients, with the cervical cord being the next most common location. Histological examination demonstrated the closely apposed vascular channels characteristic of cavernous angiomas. While most vascular channels were thickened and hyalinized, three angiomas had foci of small vessels resembling a capillary angioma. The available data suggest that surgical management of intramedullary angiomas should attempt complete extirpation. The lesions are often well circumscribed with a glial plane between the lesion and normal cord. However, spinal cord angiomas, unlike cranial lesions, have little room for enlargement before devastating symptoms occur. We achieved complete excision in all of our patients, who are all ambulatory and have improved symptoms. Little, if any, increase in neurological deficit was caused by myelotomy and lesion excision. These spinal lesions should be considered for surgery early, before repeated hemorrhage or enlargement can occur.

摘要

脊髓海绵状血管瘤是一种罕见的病变,可导致严重的神经症状。我们将6例脊髓髓内海绵状血管瘤患者加入到文献中已描述的30例有31个经组织学证实病变的患者中。36例患者出现了四种临床表现类型:1)神经功能恶化的离散发作,发作之间有不同程度的恢复(13例患者);2)神经功能逐渐衰退(12例患者);3)症状急性发作且迅速衰退(8例患者);4)轻度症状急性发作,随后逐渐衰退持续数周或数月(3例患者)。36例患者中,25例(69%)为女性,11例(31%)为男性。发病的高峰年龄在第四个十年。超过一半的患者胸段脊髓受累,其次最常见的部位是颈段脊髓。组织学检查显示了海绵状血管瘤特有的紧密相邻的血管通道。虽然大多数血管通道增厚并玻璃样变,但有三个血管瘤有类似于毛细血管瘤的小血管灶。现有数据表明,脊髓髓内血管瘤的手术治疗应尝试完全切除。病变通常界限清楚,在病变与正常脊髓之间有一个胶质平面。然而,与颅内病变不同,脊髓血管瘤在出现严重症状之前几乎没有增大的空间。我们所有的患者均实现了完全切除,他们都能行走且症状有所改善。脊髓切开术和病变切除术几乎没有或没有导致神经功能缺损增加。这些脊髓病变应在反复出血或增大发生之前尽早考虑手术治疗。

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