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椎管内脊髓海绵状血管畸形的临床表现、自然史和转归。

Clinical presentation, natural history and outcomes of intramedullary spinal cord cavernous malformations.

机构信息

Neurosurgery, Mayo Clinic, Rochester, New York, USA.

Neurology, Mayo Clinic, Rochester, New York, USA.

出版信息

J Neurol Neurosurg Psychiatry. 2019 Jun;90(6):695-703. doi: 10.1136/jnnp-2018-319553. Epub 2019 Feb 13.

DOI:10.1136/jnnp-2018-319553
PMID:30760644
Abstract

OBJECTIVE

There is a paucity of literature investigating the clinical course of patients with spinal intramedullary cavernous malformations (ISCMs). We present a large case series of ISCMs to describe clinical presentation, natural history and outcomes of both surgical and conservative management.

METHODS

We retrospectively reviewed the clinical course of patients diagnosed with ISCMs at our institution between 1995 and 2016. Haemorrhage was defined as clinical worsening in tandem with imaging changes visualised on follow-up MRI. Outcomes assessed included neurological status and annual haemorrhage rates.

RESULTS

A total of 107 patients met inclusion criteria. Follow-up data were available for 85 patients. While 21 (24.7%) patients underwent immediate surgical resection, 64 (75.3%) were initially managed conservatively. Among this latter group, 16 (25.0%) suffered a haemorrhage during follow-up and 11 (17.2%) required surgical resection due to interval bleeding or neurological worsening. The overall annual risk of haemorrhage was 5.5% per person year. The rate among patients who were symptomatic and asymptomatic on presentation was 9.5% and 0.8%, respectively. Median time to haemorrhage was 2.3 years (0.1-12.3). Univariate analysis identified higher ISCM size (p=0.024), history of prior haemorrhage (p=0.013) and presence of symptoms (p=0.003) as risk factors for subsequent haemorrhage. Multivariable proportional hazards analysis revealed presence of symptoms to be independently associated with haemorrhage during follow-up (HR 9.39, CI 1.86 to 170.8, p=0.013).

CONCLUSION

Large, symptomatic ISCMs appear to be at increased risk for subsequent haemorrhage. Surgery may be considered in such lesions to prevent rebleeding and subsequent neurological worsening.

摘要

目的

关于脊髓髓内海绵状血管畸形(ISCMs)患者的临床病程,文献报道较少。我们报告了一系列较大的 ISCM 病例,以描述手术和保守治疗的临床表现、自然病程和结局。

方法

我们回顾性分析了 1995 年至 2016 年期间在我院诊断为 ISCMs 的患者的临床病程。出血定义为与随访 MRI 上可见的影像学变化同时出现的临床恶化。评估的结局包括神经状态和每年出血率。

结果

共纳入 107 例患者。85 例患者有随访资料。21 例(24.7%)患者行立即手术切除,64 例(75.3%)患者行初始保守治疗。在后者组中,16 例(25.0%)在随访期间发生出血,11 例(17.2%)因间隔性出血或神经恶化需要手术切除。总的年出血风险为每人每年 5.5%。就诊时症状性和无症状性患者的出血率分别为 9.5%和 0.8%。出血的中位时间为 2.3 年(0.1-12.3)。单因素分析发现,较大的 ISCM 大小(p=0.024)、既往出血史(p=0.013)和症状存在(p=0.003)是随后出血的危险因素。多变量比例风险分析显示,症状存在与随访期间出血独立相关(HR 9.39,CI 1.86 至 170.8,p=0.013)。

结论

大的、有症状的 ISCM 似乎有更高的后续出血风险。对于此类病变,可考虑手术以防止再出血和随后的神经恶化。

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