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Chiari 1畸形患者脊髓空洞症的自发改善:病例说明

Spontaneous improvement in syringomyelia in a patient with Chiari 1 malformation: illustrative case.

作者信息

Ozturk Oyku, Cındıl Emetullah, Emmez Hakan, Kuzucu Pelin, Celtıkcı Emrah

机构信息

Departments of1Neurosurgery and.

2Radiology, Gazi University Faculty of Medicine, Ankara, Turkey.

出版信息

J Neurosurg Case Lessons. 2023 Jul 17;6(3). doi: 10.3171/CASE23210.

DOI:10.3171/CASE23210
PMID:37486887
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10555572/
Abstract

BACKGROUND

"Chiari malformation" refers to a spectrum of hindbrain abnormalities characterized by impaired cerebrospinal fluid circulation through the foramen magnum. Syringomyelia is frequently found in patients with Chiari malformation type 1. Although many theories have suggested how cerebrospinal fluid enters and makes the cystic cavity in the spinal cord, the pathogenesis of syringomyelia remains controversial. This report documents a case with spontaneous resolution of syringomyelia followed up by 3-year serial magnetic resonance imaging (MRI). These kinds of cases support a more conservative approach.

OBSERVATIONS

A 59-year-old female presented to the authors' clinic in June 2019 with a history of Chiari malformation type 1. This symptomatic patient has been followed up with serial MRI. When the last MRI was performed in August 2022, compared with previous imaging, resolution of the syringomyelia was recognized.

LESSONS

Because the natural evolution of mildly symptomatic/asymptomatic patients with syringes is unclear, these patients pose a treatment dilemma. Although surgical intervention is a widely accepted therapeutic method, a more conservative approach can be considered in cases with spontaneous resolution. Especially for patients without progressive symptoms, the surgical approach should not be considered as the first step. In view of relapses, follow-up with periodic neurological examinations and radiological imaging is preferrable.

摘要

背景

“Chiari 畸形”指的是一系列后脑异常,其特征是通过枕骨大孔的脑脊液循环受损。脊髓空洞症在 Chiari 1 型畸形患者中经常出现。尽管有许多理论提出了脑脊液如何进入并形成脊髓囊性腔隙,但脊髓空洞症的发病机制仍存在争议。本报告记录了一例脊髓空洞症自发缓解的病例,并通过连续 3 年的磁共振成像(MRI)进行随访。这类病例支持采取更为保守的方法。

观察结果

一名 59 岁女性于 2019 年 6 月到作者所在诊所就诊,有 Chiari 1 型畸形病史。这名有症状的患者通过连续 MRI 进行随访。2022 年 8 月进行最后一次 MRI 检查时,与之前的影像相比,脊髓空洞症已消失。

经验教训

由于症状轻微/无症状的脊髓空洞症患者的自然病程尚不清楚,这些患者面临治疗困境。尽管手术干预是一种被广泛接受的治疗方法,但对于自发缓解的病例可考虑采取更为保守的方法。特别是对于没有进行性症状的患者,不应将手术方法作为首选。鉴于有复发情况,定期进行神经学检查和影像学检查进行随访更佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3259/10555572/61fc76534642/CASE23210f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3259/10555572/61fc76534642/CASE23210f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3259/10555572/61fc76534642/CASE23210f1.jpg

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本文引用的文献

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Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case.间歇性脊髓空洞症:突出脊髓空洞症复杂的病理生理学。病例说明。
J Neurosurg Case Lessons. 2021 Sep 13;2(11):CASE21341. doi: 10.3171/CASE21341.
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Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case.Chiari I型畸形患者自发性脊髓空洞症消退:病例说明
J Neurosurg Case Lessons. 2021 Jun 28;1(26):CASE21236. doi: 10.3171/CASE21236.
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Syringomyelia: diagnosis and management.脊髓空洞症:诊断与治疗。
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Spontaneous Resolution of Syringomyelia with a 16-Year Serial Magnetic Resonance Imaging Follow-Up: A Case Report and Literature Review.脊髓空洞症的 16 年磁共振成像随访自然缓解:病例报告及文献复习。
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