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Chiari I型畸形患者自发性脊髓空洞症消退:病例说明

Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case.

作者信息

Gallo Elizabeth, Rahmathulla Gazanfar, Rao Dinesh, Tavanaiepour Kourosh, Tavanaiepour Daryoush

机构信息

Department of Neurosurgery, and.

Division of Neuroradiology, University of Florida, College of Medicine, Jacksonville, Florida.

出版信息

J Neurosurg Case Lessons. 2021 Jun 28;1(26):CASE21236. doi: 10.3171/CASE21236.

DOI:10.3171/CASE21236
PMID:35854904
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9245757/
Abstract

BACKGROUIND

Chiari malformations include a spectrum of congenital hindbrain herniation syndromes. In patients with the most common subtype, Chiari malformation Type I, 50% to 75% develop a syrinx. The pathogenesis of syringomyelia is not well understood, with multiple theories outlined in the literature. Although the presence of a syrinx in a patient with Chiari malformation is generally accepted as an indication for surgical intervention, there are documented cases of spontaneous resolution that support a more conservative approach to management.

OBSERVATIONS

The authors reported a case of spontaneous resolution of a cervical syrinx in an adult with an unchanged Chiari malformation.

LESSONS

Given the possibility of spontaneous resolution over time, the authors believe a more conservative approach of observation with periodic surveillance, magnetic resonance imaging, and neurological examination should be considered in the management of a patient with a Chiari malformation and associated syringomyelia.

摘要

背景

Chiari畸形包括一系列先天性后脑疝综合征。在最常见的亚型Chiari I型患者中,50%至75%会出现脊髓空洞症。脊髓空洞症的发病机制尚不完全清楚,文献中概述了多种理论。虽然Chiari畸形患者出现脊髓空洞症通常被认为是手术干预的指征,但有记录显示存在自发缓解的病例,这支持了一种更保守的治疗方法。

观察结果

作者报告了一例成年患者颈段脊髓空洞症自发缓解且Chiari畸形无变化的病例。

经验教训

鉴于随着时间推移有自发缓解的可能性,作者认为在Chiari畸形及相关脊髓空洞症患者的治疗中,应考虑采用更保守的观察方法,定期进行监测、磁共振成像和神经学检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43e1/9245757/b84de9180d59/CASE21236f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43e1/9245757/7128537db18e/CASE21236f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43e1/9245757/b84de9180d59/CASE21236f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43e1/9245757/7128537db18e/CASE21236f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43e1/9245757/b84de9180d59/CASE21236f2.jpg

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World Neurosurg. 2019 Oct;130:432-438. doi: 10.1016/j.wneu.2019.07.138. Epub 2019 Jul 24.
2
Chiari I malformation and altered cerebrospinal fluid dynamics-the highs and the lows.Chiari I型畸形与脑脊液动力学改变——起伏情况
Childs Nerv Syst. 2019 Oct;35(10):1711-1717. doi: 10.1007/s00381-019-04233-w. Epub 2019 Jun 16.
3
Management of Chiari malformations: opinions from different centers-a review.
Chiari畸形的管理:不同中心的观点——综述
Childs Nerv Syst. 2019 Oct;35(10):1869-1873. doi: 10.1007/s00381-019-04176-2. Epub 2019 May 24.
4
Posterior fossa decompression for Chiari malformation type I: clinical and radiological presentation, outcome and complications in a retrospective series of 105 procedures.后颅窝减压术治疗 Chiari 畸形 I 型:105 例回顾性系列的临床和影像学表现、结果和并发症。
Acta Neurol Belg. 2019 Jun;119(2):245-252. doi: 10.1007/s13760-019-01086-7. Epub 2019 Feb 8.
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Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients.Chiari 畸形伴或不伴脊髓空洞症的手术治疗:177 例成年患者的经验。
J Neurosurg. 2013 Feb;118(2):232-42. doi: 10.3171/2012.10.JNS12305. Epub 2012 Nov 23.
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Spontaneous resolution of syrinx: report of two cases in adults with Chiari malformation.脊髓空洞自然缓解:两例成人 Chiari 畸形伴发脊髓空洞病例报告。
Neurol Sci. 2012 Apr;33(2):339-41. doi: 10.1007/s10072-011-0670-9. Epub 2011 Jun 28.
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Pathogenesis of syringomyelia associated with Chiari type 1 malformation: review of evidences and proposal of a new hypothesis.与 Chiari Ⅰ 型畸形相关的脊髓空洞症的发病机制:证据回顾与新假说的提出。
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