病例报告:1例2型神经纤维瘤病患者前庭神经鞘瘤切除术后对侧进展。

Neurosurgery practice Pub Date : 2024-01-29 eCollection Date: 2024-03-01 DOI:10.1227/neuprac.0000000000000082
Ihika Rampalli, Collin William English, Tyler Lazaro, Benjamin Daniel Lovin, Sungho Lee, Andrew Tsao Huang, Ali Jalali, Kathleen Kelly Gallagher, Jacob J Mandel, Alex Daniel Sweeney, Akash J Patel
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引用次数: 0

摘要

背景和重要性:双侧前庭神经鞘瘤(VS)是2型神经纤维瘤病(NF2)的一个显著特征,会导致患者生活质量严重下降。这些神经鞘瘤的生长速度是可变的,早期发现增加的生长是改善结果的基础。临床表现:17岁男性,NF2合并双侧VS,左侧VS切除术后不到1个月,表现为完全性右侧面瘫和突发性右侧听力丧失。影像学显示右侧VS快速生长并瘤内出血,患者行紧急手术切除,症状有所改善。讨论:已有文献表明,术后较长时间出现类似现象;然而,没有人表现出如此迅速的症状。我们探讨了这种表现的多种病因,包括原发性切除术后的突然减压、旁分泌因素和先前放疗引起的血管肥大。结论:NF2患者行VS切除术后可能伴对侧VS快速生长,导致严重的症状表现。应告知患者这种风险,以便及早发现和干预。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Case Report: Contralateral Progression of a Vestibular Schwannoma After Resection in a Patient with Neurofibromatosis Type 2.

Background and importance: Bilateral vestibular schwannomas (VS) are a distinctive feature of neurofibromatosis type 2 (NF2) that result in a serious reduction in the quality of life for patients. The growth rates of these schwannomas are variable, and the early detection of increased growth is fundamental for improving outcomes.

Clinical presentation: A 17-year-old man with NF2 and bilateral VS presented with complete right facial paralysis and sudden right hearing loss less than 1 month after resection of a left VS. Imaging revealed rapid growth of the right VS with intratumoral hemorrhage, and the patient underwent urgent surgical resection with some improvement in symptoms.

Discussion: Existing literature indicates a similar phenomenon after a longer post-operative period; however, none have shown such rapid symptomatology. Multiple etiologies were explored for this presentation, including the sudden decompression from the primary resection, paracrine factors, and hypervascularity from prior radiation.

Conclusion: VS resection in a patient with NF2 may be followed by rapid growth in the contralateral VS, leading to severe symptom presentation. Patients should be counseled regarding this risk to enable early detection and intervention.

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