PURPOSE To review the literature about the acute presentation of Chiari 1 malformation in children, with a focus on acute cervical cord syndromes with impairment of the cortico-spinal tract. To analyze the possible precipitating factors and the pathophysiology of the acute onset. METHOD Illustrative case presentation with literature review. RESULTS The literature includes just a few dozens of children with acute presentation of Chiari 1 malformation. The more frequent presentations consist of oropharyngeal dysfunction, cranial nerve impairment, high intracranial pressure, peripheral motor deficit. Acute impairment of cervical long tracts is very rare and we could find only 16 previously reported cases of cervical cord impairment with quadriparesis or hemiparesis. CONCLUSIONS Nowadays, a lot of asymptomatic Chiari 1 malformations are frequently observed owing to the wide diffusion of magnetic resonance imaging. This raised the question about the management of these patients. Despite severe and even lethal manifestations being reported in previously asymptomatic patients, the absolute rarity of acute deterioration does not justify prophylactic surgery. The diagnosis of Chiari malformation may be initially difficult in patients with isolated, acute, and unusual presentations but physicians should bear in mind its possibility, because prompt cranio-cervical decompression may be decisive.

中文翻译：

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儿童Chiari 1畸形的急性表现。

目的回顾儿童Chiari 1畸形的急性表现的文献，重点研究急性脊髓脊髓综合征伴皮质脊髓束损伤。分析可能的诱发因素和急性发作的病理生理。方法案例分析与文献综述。结果文献仅包括数十例Chiari 1畸形的急性表现儿童。较常见的表现包括口咽功能障碍，颅神经损伤，高颅内压，周围运动障碍。子宫颈长束的急性损伤非常罕见，我们只能发现16例先前报道的伴有四肢瘫痪或偏瘫的颈髓损伤病例。结论如今，由于磁共振成像的广泛扩散，经常观察到许多无症状的Chiari 1畸形。这就提出了有关这些患者管理的问题。尽管在先前无症状的患者中报告了严重甚至致命的表现，但急性恶化的绝对罕见性不能作为预防性手术的依据。对于有独立，急性和不正常表现的患者，Chiari畸形的诊断最初可能很困难，但是医生应牢记其可能性，因为及时进行颅颈减压可能是决定性的。急性恶化的绝对稀有性不能作为预防性手术的依据。对于有独立，急性和不正常表现的患者，Chiari畸形的诊断最初可能很困难，但是医生应牢记其可能性，因为及时进行颅颈减压可能是决定性的。急性恶化的绝对稀有性不能作为预防性手术的依据。对于有独立，急性和不正常表现的患者，Chiari畸形的诊断最初可能很困难，但是医生应牢记其可能性，因为及时进行颅颈减压可能是决定性的。