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多学科方法修复神经纤维瘤病 1 型大型胸腹部脑脊膜膨出;文献系统回顾及病例报告。

Multidisciplinary approach for repair of a large ventral thoracic meningocele in neurofibromatosis-1; a systematic review of the literature and case report.

机构信息

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Department of Plastic Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

出版信息

Clin Neurol Neurosurg. 2021 Nov;210:106996. doi: 10.1016/j.clineuro.2021.106996. Epub 2021 Oct 22.

Abstract

Thoracic meningoceles and dural ectasia are less commonly recognized manifestations of neurofibromatosis 1 (NF1). Rarely, large thoracic meningoceles may become compressive and lead to respiratory compromise secondary to lung compression. Surgical goals aim to increase lung aeration through decreasing the size of the meningocele through shunting, excision or repair of the meningocele, and varying degrees of dural tube reconstruction. There is no agreement on the best approach for large, symptomatic meningoceles. Here, we discuss the case of a 41-year-old woman with NF1 who presented with dyspnea and enlargement of a large, 19 cm thoracic meningocele. A multidisciplinary team of thoracic, plastic, and neurological surgery participated in the operation to excise the meningocele and reconstruct the dural tube without the need for subsequent shunting of spinal fluid. We also systematically review the literature on thoracic meningoceles in NF1 to understand the optimal treatment of this pathology.

摘要

胸脑膜膨出和硬脑膜扩张是神经纤维瘤病 1 型(NF1)较少见的表现。极少数情况下,大型胸脑膜膨出可能会压迫肺部,导致肺部压缩引起呼吸功能受损。手术目标旨在通过分流、切除或修复脑膜膨出,以及不同程度的硬脑膜管重建,减少脑膜膨出的大小,从而增加肺部通气。对于大型、有症状的脑膜膨出,目前尚无最佳治疗方法的共识。在此,我们讨论了一位 41 岁女性 NF1 患者,因呼吸困难和 19cm 大型胸脑膜膨出而就诊。胸外科、整形外科和神经外科的多学科团队参与了手术,切除脑膜膨出并重建硬脑膜管,无需随后进行脑脊液分流。我们还系统地回顾了 NF1 中胸脑膜膨出的文献,以了解该病理的最佳治疗方法。

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