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1型神经纤维瘤病合并胸腰段脊膜膨出。附2例报告并文献复习。

Thoracic and lumbar meningocele in neurofibromatosis type 1. Report of two cases and review of the literature.

作者信息

Rainov N G, Heidecke V, Burkert W

机构信息

Neurosurgical Department, Martin Luther University, Halle/Saale, Fed. Rep. of Germany.

出版信息

Neurosurg Rev. 1995;18(2):127-34. doi: 10.1007/BF00417670.

Abstract

We report two cases of ventrolateral thoracic and lumbar meningoceles associated with neurofibromatosis type 1, and present a review of the literature. Thoracic and lumbar meningoceles are rare lesions which are associated with neurofibromatosis in 60% to 85% of cases. Large meningoceles may cause pain, neurological signs, or respiratory complaints when located intrathoracically. Surgery is indicated in such cases, as well as when small meningoceles enlarge with time. The etiology of meningoceles in neurofibromatosis remains controversial. The authors postulate that ventrolateral lesions of the thoracic and lumbar spine are secondary to congenital mesodermal dysplasia and hypoplastic bone changes. MRI is the diagnostic method of choice, since it most adequately visualizes the pathological anatomy of the spinal cord, meninges, and adjacent structures in every sectional plane. CT scans are particularly useful in assessing the spinal bony changes.

摘要

我们报告了两例与1型神经纤维瘤病相关的胸腰段腹外侧脊膜膨出病例,并对相关文献进行了综述。胸段和腰段脊膜膨出是罕见病变,60%至85%的病例与神经纤维瘤病相关。大型脊膜膨出位于胸腔内时可引起疼痛、神经体征或呼吸方面的主诉。此类病例以及小型脊膜膨出随时间增大时均需手术治疗。神经纤维瘤病中脊膜膨出的病因仍存在争议。作者推测胸腰椎的腹外侧病变继发于先天性中胚层发育异常和骨发育不全改变。MRI是首选的诊断方法,因为它能在每个断面最充分地显示脊髓、脑膜及相邻结构的病理解剖。CT扫描在评估脊柱骨质改变方面特别有用。

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