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1型神经纤维瘤病中脊柱畸形与硬脊膜扩张的关联。

The association of spinal deformity with dural ectasia in neurofibromatosis type 1.

作者信息

Shah Savan, George K Joshi

机构信息

University of Manchester, Manchester, UK.

Department of Neurosurgery, Salford Royal NHS Foundation Trust, Salford, UK.

出版信息

Br J Neurosurg. 2019 Dec;33(6):620-623. doi: 10.1080/02688697.2019.1673313. Epub 2019 Oct 4.

Abstract

To establish the prevalence of dural ectasia in patients with complex neurofibromatosis type-1 (NF-1) and its association with spinal deformities that manifest alongside. Analysis of the neuroradiological notes from 24 months of multidisciplinary team meetings for patients with complex NF-1 (equating to 378 patients). Review of the MRI scans of all patients with dural ectasia with each scan graded using a novel grading system. A total of 38 of the 378 patients were found to have dural ectasia (10.05%). The median age of these 38 patients was 36 years ranging from 18 to 64. The male: female ratio was 16:22. In all, 90.9% of patients with a 'major' form of dural ectasia had a concurrent spinal deformity present compared to 18.18% of patients with a minor form. The more severe the dural ectasia, the greater the likelihood a concurrent deformity with NF-1. The vertebral bodies and pedicles are more commonly involved than the posterior elements.

摘要

确定复杂1型神经纤维瘤病(NF-1)患者硬脊膜扩张的患病率及其与同时出现的脊柱畸形的关联。分析复杂NF-1患者24个月多学科团队会议的神经放射学记录(相当于378例患者)。回顾所有硬脊膜扩张患者的MRI扫描,每次扫描使用一种新的分级系统进行分级。378例患者中共有38例发现有硬脊膜扩张(10.05%)。这38例患者的中位年龄为36岁,范围在18至64岁之间。男女比例为16:22。总体而言,“重度”硬脊膜扩张形式的患者中有90.9%同时存在脊柱畸形,而轻度形式的患者中这一比例为18.18%。硬脊膜扩张越严重,同时合并NF-1相关畸形的可能性就越大。椎体和椎弓根比后部结构更常受累。

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