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表现为面神经麻痹的管内型前庭神经鞘瘤。

Intracanalicular vestibular schwannomas presenting with facial nerve paralysis.

作者信息

Mastronardi Luciano, Cacciotti Guglielmo, Roperto Raffaele

机构信息

Division of Neurosurgery, San Filippo Neri Hospital, Rome, 00198, Italy.

出版信息

Acta Neurochir (Wien). 2018 Apr;160(4):689-693. doi: 10.1007/s00701-018-3498-8. Epub 2018 Feb 26.

Abstract

BACKGROUND

To describe the surgical management and postoperative course of two patients presenting with facial nerve (FN) paralysis as one of the presenting symptoms of small intracanalicular vestibular schwannomas (VS).

METHODS

Among 153 patients operated for VS since September 2010 to August 2017, two adult female patients presented with rapidly progressive hearing decrease, vestibular symptoms, and FN paralysis (House-Brackmann grades III and IV, respectively). In both cases, c.e. T1-weighted magnetic resonance imaging revealed an enhancing tumor within the internal auditory canal without lateral extension beyond the fundus.

RESULTS

Retrosigmoid approach and excision of tumor showed that the origin of tumor was from the superior vestibular nerve, extrinsic to FN. Gross total tumor resection was obtained, with FN preservation. In the first case, a millimetric fragment of capsule was left because of tight adhesion on FN itself. Histopathology confirmed schwannoma. After surgery, both patients improved FN motor function.

CONCLUSIONS

Although very rarely, VS may start clinically with FN palsy, mimicking FN schwannomas and other less common pathologies. This presentation is exceptional in patients with small intracanalicular VS. Early surgical resection is the only reliable treatment for decompression of nerve, avoiding a complete and not-reversible damage, with possible postoperative FN function improvement or complete recovery.

摘要

背景

描述两名以内耳道小前庭神经鞘瘤(VS)为首发症状之一且伴有面神经(FN)麻痹的患者的手术治疗及术后病程。

方法

在2010年9月至2017年8月间接受VS手术的153例患者中,两名成年女性患者表现为快速进行性听力下降、前庭症状及FN麻痹(分别为House-Brackmann III级和IV级)。两例患者的增强T1加权磁共振成像均显示内听道内有强化肿瘤,未向外侧延伸至内听道底以外。

结果

经乙状窦后入路切除肿瘤显示,肿瘤起源于FN外侧的上前庭神经。实现了肿瘤全切除并保留了FN。第一例中,因FN本身粘连紧密而残留了毫米级的包膜碎片。组织病理学证实为神经鞘瘤。术后,两名患者的FN运动功能均有改善。

结论

尽管非常罕见,但VS可能临床上以FN麻痹为首发表现,类似FN神经鞘瘤及其他少见病变。这种表现在内耳道小VS患者中很罕见。早期手术切除是唯一可靠的神经减压治疗方法,可避免神经发生完全且不可逆的损伤,术后FN功能可能改善或完全恢复。

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