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小儿Chiari I型畸形的当前管理

Current management of pediatric chiari type 1 malformations.

作者信息

Entezami Pouya, Gooch M Reid, Poggi Jonathan, Perloff Eric, Dupin Melissa, Adamo Matthew A

机构信息

Department of Neurosurgery, Albany Medical College, Albany, NY, USA.

Department of Neurosurgery, Thomas Jefferson University, Philadelphia, PA, USA.

出版信息

Clin Neurol Neurosurg. 2019 Jan;176:122-126. doi: 10.1016/j.clineuro.2018.12.007. Epub 2018 Dec 10.

DOI:10.1016/j.clineuro.2018.12.007
PMID:30557765
Abstract

OBJECTIVES

Pediatric Chiari Type 1 Malformations (CM1) are commonly referred for neurosurgical opinion. The ideal management in children regarding surgical and radiographic decision making is not clearly delineated.

PATIENTS AND METHODS

We retrospectively reviewed our cohort of patients age 18 years and younger referred to a single neurosurgeon for CM1. Baseline MRIs of the spine were obtained. Non-operative patients had repeat imaging at 6-12 months. Patients who underwent an operation (decompression with/without duraplasty) had repeat imaging at 6 months.

RESULTS

One hundred and thirty-two patients with mean age of 10 years met inclusion criteria. All patients had post-operative symptomatic improvement. We identified 26 patients with syrinx, 8 with scoliosis, 3 with hydrocephalus, and one had tethered cord. The average tonsillar descent was 8.1 mm in the non-operative group and 11.9 mm in the operative group. Ninety-five patients were managed conservatively (72%). Thirty-seven were offered surgery (28%), and 33 patients underwent intervention; 21 with duraplasty (64%) and 12 without (36%).

CONCLUSIONS

Pediatric patients with CM1 require both clinical and radiographic follow-up. Duraplasty may be performed if decompression fails to relieve symptomatology, but is not always needed. CM1 continues to present a challenge in surgical decision making. Adhering to a treatment paradigm may help alleviate difficult decision-making.

摘要

目的

小儿 Chiari Ⅰ型畸形(CM1)通常会寻求神经外科意见。关于儿童手术和影像学决策的理想管理方法尚无明确界定。

患者与方法

我们回顾性分析了转诊至一位神经外科医生处的 18 岁及以下 CM1 患儿队列。获取了脊柱的基线磁共振成像(MRI)。非手术患者在 6 - 12 个月时进行重复成像。接受手术(减压术伴或不伴硬脑膜成形术)的患者在术后 6 个月进行重复成像。

结果

132 例平均年龄为 10 岁的患者符合纳入标准。所有患者术后症状均有改善。我们发现 26 例患者有脊髓空洞症,8 例有脊柱侧弯,3 例有脑积水,1 例有脊髓栓系。非手术组扁桃体下移平均为 8.1 毫米,手术组为 11.9 毫米。95 例患者接受保守治疗(72%)。37 例患者接受手术治疗(28%),33 例患者接受了干预;21 例进行了硬脑膜成形术(64%),12 例未进行(36%)。

结论

CM1 患儿需要临床和影像学随访。如果减压术未能缓解症状,可进行硬脑膜成形术,但并非总是必要的。CM1 在手术决策方面仍然是一个挑战。遵循治疗模式可能有助于缓解艰难的决策过程。

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The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature.儿童 Chiari 畸形 1 型和脊髓空洞症的治疗:文献综述。
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Acta Neurol Belg. 2021 Dec;121(6):1547-1554. doi: 10.1007/s13760-020-01398-z. Epub 2020 Jun 10.
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