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第四脑室海绵状血管畸形的临床表现、手术治疗及术后转归:病例报告及文献复习。

Presentation, Surgical Management, and Postoperative Outcome of a Fourth Ventricular Cavernous Malformation: Case Report and Review of Literature.

机构信息

Robert Wood Johnson Medical School-Rutgers, New Brunswick, New Jersey, USA.

Department of Neurosurgery, Rutgers-Robert Wood Johnson Medical School & University Hospital, New Brunswick, New Jersey, USA.

出版信息

World Neurosurg. 2020 May;137:78-83. doi: 10.1016/j.wneu.2020.01.185. Epub 2020 Feb 3.

Abstract

BACKGROUND

Brainstem cavernous malformations (CMs) represent dangerous clinical entities associated with high rates of rebleeding and morbidity compared with those in other locations. Particularly rare are those located within the fourth ventricle. Although fourth ventricular CMs are favorable from a surgical standpoint, there are no defined guidelines on definitive indications and optimal timing of surgery. In addition, the surgical approaches, anatomic considerations, and general observations regarding these lesions are not well reported in the literature.

CASE PRESENTATION

A 27-year-old man with a known history of a CM on the floor of the fourth ventricle presented with new cranial nerve deficits and signs of increased intracranial pressure. Imaging revealed acute bleeding from a fourth ventricular CM. The patient was urgently taken to surgery for resection. Despite a noneventful surgery which resulted in gross total resection, the patient developed a unique constellation of cranial nerve deficits postoperatively, most notably of which was eight-and-a-half syndrome.

CONCLUSIONS

CMs of the fourth ventricle are rare clinical entities that can be treated successfully with surgery. The indications for surgery may not always be clear-cut; therefore, the neurosurgeon's decision to proceed with surgery must reside on a case-by-case basis using a multifactorial approach. The location of these lesions presents unique challenges given their proximity to vital structures and the technical difficulty required. For these reasons, the resection of these lesions often results in new or persistent neurologic deficits. However, despite the associated risks, the potential benefits of surgery oftentimes outweigh the risks of the alternative.

摘要

背景

与其他部位相比,脑干海绵状血管畸形(CM)是一种危险的临床实体,其再出血率和发病率都较高。尤其罕见的是位于第四脑室的 CM。虽然从手术角度来看第四脑室 CM 是有利的,但对于手术的明确适应证和最佳时机尚无明确的指南。此外,这些病变的手术入路、解剖学考虑因素和一般观察结果在文献中报道得并不充分。

病例介绍

一名 27 岁男性,已知第四脑室底部有 CM,现出现新的颅神经功能障碍和颅内压增高的迹象。影像学显示第四脑室 CM 急性出血。患者紧急接受手术切除。尽管手术过程顺利,实现大体全切除,但患者术后出现独特的颅神经功能障碍,其中最明显的是八半综合征。

结论

第四脑室 CM 是一种罕见的临床实体,可通过手术成功治疗。手术适应证不一定很明确;因此,神经外科医生决定进行手术的决定必须根据具体情况,采用多因素方法做出。由于这些病变靠近重要结构,且需要较高的技术难度,因此其位置带来了独特的挑战。出于这些原因,切除这些病变常常导致新的或持续的神经功能障碍。然而,尽管存在相关风险,但手术的潜在益处通常超过替代方案的风险。

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