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双侧前庭神经鞘瘤的管理策略。

Management strategies for bilateral vestibular schwannomas.

作者信息

Sahu Rabi N, Mehrotra N, Tyagi I, Banerji Deepu, Jain V K, Behari Sanjay

机构信息

Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Bareli Road, Lucknow 226014, India.

出版信息

J Clin Neurosci. 2007 Aug;14(8):715-22. doi: 10.1016/j.jocn.2006.05.004.

Abstract

BACKGROUND

Bilateral vestibular schwannomas (VS) are rare. Most patients in India present late in the course of illness with large tumors and disabling deafness. Clinical presentation and management goals are different from that of unilateral VS.

AIMS

To highlight the differences in clinical presentations and surgical results of bilateral VS compared to unilateral VS; and, to propose a management strategy for these tumors with reference to tumor size, extent of growth and the presence or absence of hearing impairment.

METHOD

This is a retrospective study of 16 patients with bilateral VS treated over 10 years in a tertiary referral hospital. Assessment of VIIth and VIIIth cranial nerve function, tumor size, volume and extent of growth was performed in all patients. The management strategy was based on Samii's classification of tumor extent. All patients were operated using a retromastoid suboccipital approach. Postoperative results were analyzed and compared with those of unilateral VS.

RESULTS

The mean age of presentation was 25.7 years. Hearing impairment was the commonest symptom. Headache with features of raised intracranial pressure were present in 10 (62.5%) patients. Giant tumors were present in seven (43.7%) patients; large tumors in eight (50%) and a medium-sized tumor in one (6.3%). Total tumor resection was achieved in 13 patients and subtotal resection in two. One patient was managed conservatively and followed up with serial CT scans. On the contralateral side, one large tumor required total excision. One medium sized tumor underwent sub-capsular excision in an attempt to preserve hearing. The facial nerve was anatomically preserved in seven (46.7%) patients and in one, the cochlear nerve was anatomically preserved. There was no peri-operative mortality.

CONCLUSIONS

Patients with bilateral schwannomas are younger, have larger lesions, poorer preoperative hearing and are more likely to lose either auditory and/or facial nerve function during attempted total resection of the tumor. Classifying the tumors into two groups by extent, that is, tumors extending to the cerebellopontine angle cistern (T1-T3a) and, tumors extending to or compressing the brainstem (T3b to T4b), allows the surgical strategy to be defined.

摘要

背景

双侧前庭神经鞘瘤(VS)较为罕见。印度的大多数患者在病程晚期才出现,肿瘤较大且伴有致残性耳聋。其临床表现和治疗目标与单侧VS不同。

目的

突出双侧VS与单侧VS在临床表现和手术结果上的差异;并根据肿瘤大小、生长范围以及是否存在听力障碍,为这些肿瘤提出一种治疗策略。

方法

这是一项对一家三级转诊医院10年间治疗的16例双侧VS患者的回顾性研究。对所有患者进行了第VII和第VIII颅神经功能、肿瘤大小、体积和生长范围的评估。治疗策略基于萨米(Samii)对肿瘤范围的分类。所有患者均采用乳突后枕下入路进行手术。分析术后结果并与单侧VS的结果进行比较。

结果

患者的平均就诊年龄为25.7岁。听力障碍是最常见的症状。10例(62.5%)患者出现伴有颅内压升高特征的头痛。7例(43.7%)患者为巨大肿瘤;8例(50%)为大肿瘤,1例(6.3%)为中等大小肿瘤。13例患者实现了肿瘤全切,2例为次全切除。1例患者接受保守治疗并通过连续CT扫描进行随访。在对侧,1例大肿瘤需要全切。1例中等大小肿瘤进行了包膜下切除以试图保留听力。7例(46.7%)患者的面神经在解剖学上得以保留,1例患者的蜗神经在解剖学上得以保留。无围手术期死亡。

结论

双侧神经鞘瘤患者更年轻,病变更大,术前听力更差,在试图全切肿瘤时更有可能失去听觉和/或面神经功能。根据肿瘤范围将肿瘤分为两组,即延伸至桥小脑角池的肿瘤(T1 - T3a)和延伸至或压迫脑干的肿瘤(T3b至T4b),有助于确定手术策略。

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