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单纯后颅窝减压能否有效治疗甚至伴有严重脊髓空洞症的Ⅰ型Chiari畸形患者?病例说明。

Can posterior fossa decompression alone effectively treat Chiari malformation type I patients with even severe syringes? Illustrative cases.

作者信息

Mohammad Amro H, Lacroix Caroline, Saint-Martin Christine, Dudley Roy W R

机构信息

Faculty of Medicine and Health Sciences, McGill University, Montreal, Quebec, Canada.

Department of Medical Imaging, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada.

出版信息

J Neurosurg Case Lessons. 2025 Mar 31;9(13). doi: 10.3171/CASE24777.

Abstract

BACKGROUND

The benefit of posterior fossa decompression (PFD) alone (without duraplasty) for Chiari malformation type I (CM-I) remains controversial. Many neurosurgeons still open the dura when a syrinx is present, particularly for what appears to be a severe syrinx. Based on the existing literature, the authors hypothesized that even a severe syrinx would respond well to a PFD alone.

OBSERVATIONS

In a proof-of-principle study, the authors prospectively included 4 patients with CM-I, aged 3-15 years, with severe symptomatic syringes (spanning multiple cervical/thoracic levels and/or focally expanding the spinal cord) to undergo PFD with pre- and postdissection intraoperative MRI (iMRI). In all cases, same-day postdissection iMRI cine images showed enhanced CSF flow compared with predissection iMRI findings. Three of the 4 patients showed early syrinx and symptom improvement (≤ 6 weeks); all showed improvements at long-term follow-up (≥ 2 years). The mean hospital length of stay (LOS) was 1.25 days, with no complications or repeat surgery needed, and the mean follow-up was 37.5 months.

LESSONS

CM-I can be treated via PFD alone without duraplasty, even with a severe syrinx. Improved CSF flow is achieved intraoperatively, and the syrinx can significantly decrease over a 6-week to 1-year period postoperatively. Additional benefits include a low risk of complications and a short LOS. https://thejns.org/doi/10.3171/CASE24777.

摘要

背景

单纯后颅窝减压术(PFD,不进行硬脑膜成形术)治疗Ⅰ型Chiari畸形(CM-Ⅰ)的益处仍存在争议。许多神经外科医生在存在脊髓空洞症时仍会打开硬脑膜,尤其是对于看起来严重的脊髓空洞症。基于现有文献,作者推测即使是严重的脊髓空洞症,单独进行PFD也会有良好效果。

观察结果

在一项原理验证研究中,作者前瞻性纳入了4例年龄在3至15岁的CM-Ⅰ患者,这些患者患有严重的有症状脊髓空洞症(跨越多个颈/胸段水平和/或局部使脊髓扩张),接受了PFD手术,并在解剖前后进行术中磁共振成像(iMRI)。在所有病例中,与解剖前iMRI结果相比,解剖后当日的iMRI电影图像显示脑脊液流动增强。4例患者中有3例早期脊髓空洞症和症状改善(≤6周);所有患者在长期随访(≥2年)时均有改善。平均住院时间(LOS)为1.25天,无并发症或无需再次手术,平均随访时间为37.5个月。

经验教训

CM-Ⅰ即使伴有严重脊髓空洞症,也可单独通过PFD治疗而无需硬脑膜成形术。术中可实现脑脊液流动改善,术后6周至1年期间脊髓空洞症可显著缩小。其他益处包括并发症风险低和住院时间短。https://thejns.org/doi/10.3171/CASE24777

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1954/11959644/61a1071e6eba/CASE24777_figure_1.jpg

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