Rosenblum B, Oldfield E H, Doppman J L, Di Chiro G
Clinical Neurosurgery Section, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, Maryland.
J Neurosurg. 1987 Dec;67(6):795-802. doi: 10.3171/jns.1987.67.6.0795.
The medical records and arteriograms of 81 patients with spinal arteriovenous malformations (AVM's) were reviewed, and the vascular lesions were classified as dural arteriovenous (AV) fistulas or intradural AVM's. Intradural AVM's were further classified as intramedullary AVM's (juvenile and glomus types) and direct AV fistulas, which were extramedullary or intramedullary in location. Dural AV fistulas were defined as being supplied by a dural artery and draining into spinal veins via an AV shunt in the intervertebral foramen. Intramedullary AVM's were defined as having the AV shunt contained at least partially within the cord or pia and receiving arterial supply by medullary arteries. Of the 81 patients, 27 (33%) had dural AV fistulas and 54 (67%) had intradural AVM's. Several dissimilarities in clinical and radiographic findings of the two subgroups were evident. The patients with intramedullary AVM's were younger; the age at onset of symptoms averaged 27 years compared to 49 years for dural AV fistulas. The most common initial symptom associated with dural AV fistulas was steadily progressive paresis, whereas hemorrhage was the most common presenting symptom in cases of intramedullary lesions. No patients with dural AV fistulas had subarachnoid hemorrhage. Activity exacerbated symptoms more frequently in patients with dural lesions. Associated vascular anomalies occurred only in cases of intradural AVM's. In 96% of the dural lesions the AV nidus was in the low thoracic or lumbar region; in only 15% did the intercostal or lumbar arteries supplying the AVM also provide a medullary artery which supplied the spinal cord. In contrast, most intradural AVM's (84%) were in the cervical or thoracic segments of the spinal cord and all of them were supplied by medullary arteries. Transit of contrast medium through the intradural AVM's was rapid in 80% of cases, suggesting high-flow lesions. Forty-four percent of the patients with AVM's of the spinal cord had associated saccular arterial or venous spinal aneurysms. No dural AV fistulas displayed these characteristics. A good outcome occurred in 88% of patients with dural AV fistulas after nidus obliteration, while 49% of patients with intramedullary AVM's did well after surgery or embolization. These findings suggest that dural and intradural AVM's differ in etiology (acquired vs. congenital) and that they have different pathophysiology, radiographic findings, clinical presentation, and response to treatment.
回顾了81例脊髓动静脉畸形（AVM）患者的病历和动脉造影，血管病变分为硬脑膜动静脉（AV）瘘或硬脊膜内AVM。硬脊膜内AVM进一步分为髓内AVM（青少年型和球状型）和直接AV瘘，后者位于髓外或髓内。硬脑膜AV瘘定义为由硬脑膜动脉供血，并通过椎间孔的AV分流引流至脊髓静脉。髓内AVM定义为AV分流至少部分包含在脊髓或软膜内，并由髓动脉供血。81例患者中，27例（33%）有硬脑膜AV瘘，54例（67%）有硬脊膜内AVM。两个亚组在临床和影像学表现上有一些明显的差异。髓内AVM患者较年轻；症状出现时的平均年龄为27岁，而硬脑膜AV瘘患者为49岁。与硬脑膜AV瘘相关的最常见初始症状是进行性肌无力，而出血是髓内病变最常见的首发症状。硬脑膜AV瘘患者无蛛网膜下腔出血。活动使硬脑膜病变患者的症状更频繁加重。相关血管异常仅发生在硬脊膜内AVM病例中。96%的硬脑膜病变中AV病灶位于胸段下部或腰段；仅15%供应AVM的肋间动脉或腰动脉也提供供应脊髓的髓动脉。相比之下,大多数硬脊膜内AVM（84%）位于脊髓颈段或胸段，且均由髓动脉供血。80%的病例中造影剂通过硬脊膜内AVM的速度很快，提示为高流量病变。44%的脊髓AVM患者伴有囊状动脉或静脉脊髓动脉瘤。硬脑膜AV瘘无这些特征。硬脑膜AV瘘患者在病灶闭塞后88%预后良好，而髓内AVM患者在手术或栓塞后49%预后良好。这些发现表明，硬脑膜和硬脊膜内AVM在病因（后天性与先天性）上不同，且它们具有不同的病理生理学、影像学表现、临床表现和对治疗的反应。
