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儿童 Chiari I 畸形的各种表现及术后改善。

Various manifestation of Chiari I malformation in children and improvement after surgery.

机构信息

Neurosurgical department, UMC Maribor, 2000 Maribor, Slovenia.

Neurosurgical section of Traumatological department, General Hospital Celje, 3000 Celje, Slovenia.

出版信息

J Integr Neurosci. 2022 May 30;21(4):100. doi: 10.31083/j.jin2104100.

DOI:10.31083/j.jin2104100
PMID:35864752
Abstract

INTRODUCTION

Chiari I malformation is defined as caudal displacement of the cerebellar tonsils into the foramen magnum. The most commonly associated finding is cervical syringomyelia. The most common presenting symptom is occipital and upper cervical pain. The incidence of syringomyelia is between 30% and 70%. Surgery is recommended for symptomatic patients. The main purpose of the study is to present the data of children with Chiari type I malformation that were treated surgically in low-volume center and to describe some atypical presentations in children.

MATERIALS AND METHODS

At University Medical Centre Maribor, Slovenia, we performed a retrospective study of children with Chiari type I malformation that were treated surgically in the period from 2012 until 2021. The indication for surgery was symptomatic Chiari type I malformation. Suboccipital decompression with laminectomy of at least C1 (in one case also C2) with splitting of dura or duraplasty was performed. In a few cases coagulation of both tonsillar tips was necessary to achieve sufficient decompression and restoration of adequate cerebrospinal fluid (CSF) flow.

RESULTS

Ten children (under 18 years of age) underwent surgery for Chiari I malformation. Four patients had atypical presentation. In nine patients there was improvement after surgery. Complication rate was zero with no revision surgery. In one case suboccipital headache persisted. In all cases with preoperative syringomyelia it improved after surgery. Scoliosis improved in two out of three cases. There was no mortality after the procedure.

DISCUSSION

When patients with Chiari I malformation become symptomatic, it is recommended to perform surgery as soon as possible. Comparing the techniques; dura-splitting technique has advantages of lesser operation duration, lesser intraoperative bleeding and lower complication rates than duraplasty.

CONCLUSIONS

The majority of patients with Chiari I malformation improve after surgery. Surgical procedure is safe with very low morbidity and mortality. Surgical technique must be an individualized patient tailored choice.

摘要

介绍

Chiari I 畸形定义为小脑扁桃体经枕大孔向颅后窝移位。最常见的相关发现是颈段脊髓空洞症。最常见的首发症状是枕部和上颈段疼痛。脊髓空洞症的发病率为 30%至 70%之间。对于有症状的患者推荐手术治疗。本研究的主要目的是报告在低容量中心接受手术治疗的 Chiari I 型畸形患儿的数据,并描述儿童中一些不典型的表现。

材料和方法

在斯洛文尼亚马里博尔大学医学中心,我们对 2012 年至 2021 年期间接受手术治疗的 Chiari I 型畸形患儿进行了回顾性研究。手术指征为有症状的 Chiari I 型畸形。行枕下减压术,行至少 C1 (1 例也包括 C2)的椎板切除术,硬脑膜切开或硬脑膜成形术。在少数情况下,需要对两个扁桃体尖端进行凝固以实现足够的减压和恢复适当的脑脊液(CSF)流动。

结果

10 名儿童(年龄在 18 岁以下)因 Chiari I 畸形接受手术治疗。4 例患儿表现不典型。9 例患儿术后症状改善。无并发症,无需再次手术。1 例患者术后仍有枕下头痛。所有术前存在脊髓空洞症的患者术后均有改善。3 例脊柱侧凸中有 2 例改善。术后无死亡病例。

讨论

当 Chiari I 畸形患者出现症状时,建议尽快进行手术。比较各种技术,硬脑膜切开术与硬脑膜成形术相比,具有手术时间短、术中出血少、并发症发生率低的优点。

结论

大多数 Chiari I 畸形患者术后症状改善。手术过程安全,发病率和死亡率极低。手术技术必须根据患者个体情况进行选择。

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