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Chiari 畸形Ⅰ型硬脊膜内发现的再评估。

Reappraisal of intradural findings in Chiari malformation type I.

出版信息

Neurosurg Focus. 2023 Mar;54(3):E2. doi: 10.3171/2022.12.FOCUS22628.

Abstract

OBJECTIVE

Management of Chiari malformation type I (CM-I) requires the functional restoration of an obstructed cisterna magna. In posterior fossa decompression with duraplasty (PFDD), various intradural pathologies are suggested to alter CSF flow at the craniocervical junction and require surgical correction. However, reports of the spectrum of intraoperative intradural findings and their nuances are scarce, especially those characterizing rarer findings pertaining to the vascular structures and vascular compression.

METHODS

The authors conducted a retrospective cohort analysis of adults and children who underwent first-time PFDD for CM-I (2011-2021), with and without syringomyelia. The surgical reports and intraoperative videos were reviewed, and the frequency and nature of the intradural observations in regard to the tonsils, arachnoid, and vasculature were analyzed along with the clinical findings and surgical outcomes.

RESULTS

All 180 patients (age range 1-72 years; median [interquartile range] 24 (14-38) years; 37% of patients were children < 21 years of age) exhibited multiple intradural findings, with a median of 7 distinct concurrent observations in each patient. Novel findings not previously reported included posterior inferior communicating artery (PICA) branches compressing the neural elements at the cervicomedullary junction (26.7%). Other common findings included arachnoid adhesions (92.8%), thickening (90.6%), webs at the obex (52.2%), tonsillar gliosis (57.2%), tonsillar hypertrophy (18.3%), adhesions obstructing the foramen of Magendie (FoM) (62.2%), PICA obstruction of the FoM (17.2%), and dural scarring (87.8%). Tonsillar gliosis and intertonsillar adhesions obstructing the FoM were more common in children than adults. Tonsillar gliosis and arachnoid webs were more common among syringomyelia patients. After multivariable adjustment, none of the observed findings were independently associated with syringomyelia, preoperative symptoms, or postoperative improvement. The vast majority of patients improved postoperatively. The complication rate was low: 1.2% of patients required revision PFDD at > 3 years postoperatively, 3.6% experienced other operative complications, and 0% had CSF leaks.

CONCLUSIONS

The diversity of intradural findings and observations revealed in this study suggests that obstructive and compressive structural anomalies may be more common than previously reported among CM-I patients, both those patients with and those without syringomyelia and especially those with obstructive and compressive PICA branches. Although the authors cannot conclude that all these findings are necessarily pathological, further study may determine how they contribute to CM-I pathology and symptomatology in the setting of a compromised cisterna magna.

摘要

目的

Chiari 畸形 I 型(CM-I)的治疗需要恢复受阻的小脑延髓池的功能。在后颅窝减压伴硬脑膜成形术中(PFDD),各种硬脑膜内病变被认为会改变颅颈交界处的 CSF 流动,并需要手术矫正。然而,术中硬脑膜内发现的报告及其细微差别很少,特别是与血管结构和血管压迫有关的罕见发现。

方法

作者对 2011 年至 2021 年间首次接受 CM-I 的成人和儿童进行了回顾性队列分析,这些患者均接受了 PFDD 治疗,其中部分患者合并脊髓空洞症。对手术报告和术中录像进行了回顾,分析了与扁桃体、蛛网膜和血管有关的硬脑膜内观察结果的频率和性质,以及临床发现和手术结果。

结果

所有 180 例患者(年龄 1-72 岁;中位数[四分位距]24(14-38)岁;37%的患者年龄<21 岁)均表现出多种硬脑膜内发现,每名患者中有中位数为 7 种同时存在的不同观察结果。以前未报道过的新发现包括压迫颈髓交界处神经结构的小脑后下动脉(PICA)分支(26.7%)。其他常见发现包括蛛网膜粘连(92.8%)、增厚(90.6%)、蚓部蛛网膜炎(52.2%)、扁桃体神经胶质增生(57.2%)、扁桃体肥大(18.3%)、阻碍正中孔(Magendie 孔)的粘连(62.2%)、PICA 阻碍正中孔(17.2%)和硬脑膜瘢痕(87.8%)。扁桃体神经胶质增生和扁桃体间粘连阻碍正中孔在儿童中比在成人中更常见。扁桃体神经胶质增生和蛛网膜网在脊髓空洞症患者中更常见。多变量调整后,观察到的任何发现均与脊髓空洞症、术前症状或术后改善无关。绝大多数患者术后均有改善。并发症发生率低:1.2%的患者在术后>3 年需要再次进行 PFDD 翻修,3.6%的患者出现其他手术并发症,0%的患者出现 CSF 漏。

结论

本研究揭示了硬脑膜内发现和观察结果的多样性,表明在伴有和不伴有脊髓空洞症的 CM-I 患者中,阻塞性和压迫性结构异常可能比以前报道的更为常见,尤其是伴有阻塞性和压迫性 PICA 分支的患者。尽管作者无法得出所有这些发现都是病理性的结论,但进一步的研究可能会确定它们如何导致 CM-I 病理和症状学在小脑延髓池受压的情况下发生变化。

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