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脑室内脑内海绵状血管畸形:12 例患者系列病例和文献复习。

Intraventricular cerebral cavernomas: a series of 12 patients and review of the literature.

机构信息

Department of Neurosurgery, Helsinki University Central Hospital, Helsinki, Finland.

出版信息

J Neurosurg. 2010 Jan;112(1):140-9. doi: 10.3171/2009.3.JNS081693.

Abstract

OBJECT

Intraventricular cavernomas (IVCs) occur in only 2-10% of patients with cerebral cavernomas. Reports concerning IVC are scarce and are limited mostly to sporadic case reports. In this paper, the authors present a series of 12 patients with IVCs that were treated at a single neurosurgical department. In addition, the authors reviewed the literature.

METHODS

All clinical data were analyzed retrospectively. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale. The authors also conducted a PubMed search and found 77 cases of IVC.

RESULTS

The patients' median age was 47 years, and the male/female ratio was 2:1. A cavernoma occurred in the lateral ventricle in 6 patients, in another 5 it was in the fourth ventricle, and 1 had a lesion in the third ventricle. Almost all patients presented with acute headache on admission and in more than half, the symptoms were related to cavernoma bleeding. In total, 8 rebleedings occurred in 5 patients during a median of 0.4 years. Three patients with a cavernoma of the fourth ventricle presented with a cranial nerve deficit. In 8 cases, a cavernoma was surgically treated an average of 1.3 years after the diagnosis. Only 1 patient underwent surgery in the acute phase after a major intraventricular/intracerebral hemorrhage. The median follow-up time was 2 years. No patient was lost to follow-up, and no patient died. In total, on follow-up 9 patients improved and 3 had a persistent neurological deficit, of which 2 existed before surgery.

CONCLUSIONS

In the present series, the IVCs had a high tendency for rehemorrhage. Surgery is advocated when hemorrhages are frequent, and the mass effect causes progressive neurological deficits. Microsurgical removal of the IVC is safe, but in the fourth ventricle it can carry increased risk for cranial nerve deficits.

摘要

目的

脑室内海绵状血管畸形(IVC)仅占脑海绵状血管畸形患者的 2-10%。关于 IVC 的报告很少,且大多局限于散发病例报告。本文作者介绍了在一个神经外科治疗的 12 例 IVC 患者。此外,作者还回顾了文献。

方法

所有临床数据均进行回顾性分析。向所有患者发送随访问卷。使用格拉斯哥预后量表评估结果。作者还进行了 PubMed 搜索,发现了 77 例 IVC 病例。

结果

患者的中位年龄为 47 岁,男女比例为 2:1。6 例患者的海绵状血管畸形位于侧脑室,5 例位于第四脑室,1 例位于第三脑室。几乎所有患者入院时均表现为急性头痛,超过一半的患者症状与海绵状血管畸形出血有关。在中位时间 0.4 年内,5 例患者共发生 8 次再出血。3 例第四脑室海绵状血管畸形患者出现颅神经缺损。8 例患者在诊断后平均 1.3 年接受了手术治疗。仅 1 例在发生严重脑室内/脑内出血后在急性期接受了手术。中位随访时间为 2 年。无患者失访,无患者死亡。随访时,9 例患者改善,3 例患者存在持续性神经功能缺损,其中 2 例存在于手术前。

结论

在本系列中,IVC 再出血倾向较高。当出血频繁且肿块效应导致进行性神经功能缺损时,建议手术治疗。IVC 的显微镜下切除是安全的,但在第四脑室,可能会增加颅神经缺损的风险。

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