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一系列髓内脊髓动静脉畸形(II型球样畸形)的后路手术入路

The posterior approach to a series of glomus (Type II) intramedullary spinal cord arteriovenous malformations.

作者信息

Connolly E S, Zubay G P, McCormick P C, Stein B M

机构信息

Department of Neurological Surgery, The Neurological Institute, College of Physicians and Surgeons, Columbia University, New York, New York 10032, USA.

出版信息

Neurosurgery. 1998 Apr;42(4):774-85; discussion 785-6. doi: 10.1097/00006123-199804000-00057.

Abstract

OBJECTIVE

Intramedullary spinal cord arteriovenous malformations (AVMs) are comprised of those AVMs which in either glomus or nidal form are found partially or entirely within the substance of the spinal cord. An effort to subdivide these lesions into either glomus Type II or juvenile Type III spinal cord AVMs has underscored the diffuse, unresectable nature of the latter but has done little to suggest a logical management paradigm for the more well-defined, low-flow glomus lesion.

METHODS

To address this shortcoming, 15 cases of glomus Type II spinal AVMs were identified from a larger series of a variety of spinal AVMs. Computed tomography, magnetic resonance imaging, and superselective angiography rendered the anatomic diagnosis of these lesions accurate in all cases. All lesions were approached posteriorly via a standard laminectomy, with the goal of total extirpation, and all patients underwent immediate postoperative angiography to verify cure. In the vast majority of cases, patients underwent follow-up imaging to confirm the durability of this cure, and long-term outcome was determined using both a subjective quality of life assay and a functional grading scale. Age, sex, mode of presentation, and radiographic features were subsequently analyzed with respect to functional outcome.

RESULTS

Most glomus spinal AVMs presented with a rapid cascade of signs and symptoms (73%), often caused by subarachnoid hemorrhage (60%) and often leading to significant neurological deficits (47%). Initially, 14 of 15 patients (94%) were angiographically cured of their malformations. The remaining patient was neurologically improved after a 90% resection, and follow-up angiography demonstrated that the residual was thrombosed. However, not uncommonly, follow-up angiography and magnetic resonance imaging revealed recurrences, with three of the patients who underwent delayed imaging (23%) demonstrating new draining veins. Fortunately, in each case, the recurrence was asymptomatic. Therefore, the long-term cure rate (mean follow-up, 8.5 yr; range, 1-17 yr) was 80%, with no subsequent bleeding or progression of symptoms. Outcomes were generally good, with six patients (40%) demonstrating objective improvement, eight (53%) being neurologically stable, and one (7%) being worse. Sixty-six percent were independent, 20% required moderate assistance, and 14% remained entirely dependent. There were no deaths. Patients possessing lesions with large direct feeders off the anterior spinal artery and occupying a primarily anterior position in the cord fared somewhat worse, as did those with rapidly progressing symptomatology in the absence of subarachnoid hemorrhage. However, even in those with preserved or improved function, chronic pain was a significant problem, affecting one-third of all patients. Chronic pain was especially common in young women who had presented with significant preoperative pain that responded poorly to all subsequent therapeutic manipulations.

CONCLUSION

With careful evaluation of high-quality superselective angiography and the judicious use of preoperative embolization, posterior surgical approaches can deliver results comparable with those achieved for other benign intramedullary lesions in terms of long-term cure (80%), control of symptom progression (100%), and good functional outcome (86%). Despite these results, chronic dysesthetic pain syndromes are not uncommon and continue to present a significant management problem.

摘要

目的

脊髓髓内动静脉畸形(AVM)是指那些呈球囊状或巢状,部分或全部位于脊髓实质内的AVM。将这些病变细分为球囊型II型或青少年型III型脊髓AVM的努力凸显了后者弥漫性、不可切除的性质,但对于定义更明确、低流量的球囊型病变,几乎没有提出合理的治疗模式。

方法

为弥补这一不足,从一系列不同类型的脊髓AVM中确定了15例球囊型II型脊髓AVM。计算机断层扫描、磁共振成像和超选择性血管造影在所有病例中均准确做出了这些病变的解剖诊断。所有病变均通过标准椎板切除术从后方入路,目标是完全切除,所有患者术后立即进行血管造影以确认治愈。在绝大多数病例中,患者接受了随访成像以确认这种治愈的持久性,并使用主观生活质量评估和功能分级量表确定长期结果。随后分析了年龄、性别、表现方式和影像学特征与功能结果的关系。

结果

大多数球囊型脊髓AVM表现为迅速出现一系列体征和症状(73%),常由蛛网膜下腔出血引起(60%),且常导致明显的神经功能缺损(47%)。最初,15例患者中有14例(94%)在血管造影上畸形得到治愈。其余1例患者在90%切除后神经功能改善,随访血管造影显示残余部分血栓形成。然而,随访血管造影和磁共振成像显示复发并不罕见,3例接受延迟成像的患者(23%)出现了新的引流静脉。幸运的是,在每种情况下,复发均无症状。因此,长期治愈率(平均随访8.5年;范围1 - 17年)为80%,随后无出血或症状进展。结果总体良好,6例患者(40%)有客观改善,8例(53%)神经功能稳定,1例(7%)恶化。66%的患者独立,20%需要中度帮助,14%仍完全依赖他人。无死亡病例。病变有来自脊髓前动脉的大的直接供血支且主要位于脊髓前部的患者情况稍差,在无蛛网膜下腔出血的情况下症状迅速进展的患者也是如此。然而,即使在功能保留或改善的患者中,慢性疼痛也是一个严重问题,影响了所有患者的三分之一。慢性疼痛在术前疼痛严重且对所有后续治疗操作反应不佳的年轻女性中尤为常见。

结论

通过仔细评估高质量的超选择性血管造影并明智地使用术前栓塞,后路手术方法在长期治愈率(80%)、症状进展控制(100%)和良好的功能结果(86%)方面可取得与其他良性髓内病变相当的效果。尽管有这些结果,慢性感觉异常性疼痛综合征并不少见,仍然是一个重大的治疗难题。

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