Pediatric Neurosurgery, Fondazione Policlinico Gemelli IRCCS, Rome, Italy.
Università Cattolica del Sacro Cuore, Rome, Italy.
Childs Nerv Syst. 2023 Oct;39(10):2757-2769. doi: 10.1007/s00381-023-06051-7. Epub 2023 Jun 27.
In recent years thanks to the growing use of radiological assessment, Chiari I malformation became one of the major diseases for a neurosurgeon to deal with. CIM can be classified according to the extent of cerebellar tonsil tip into the foramen magnum being a protrusion over five mm considered pathological. Such a disease is a heterogeneous condition with a multifactorial pathogenetic mechanism that can subdivided into a primary and secondary form. Regardless of the form, it seems that CIM is the result of an imbalance between the volume of the braincase and its content. Acquired CIMs are secondary to conditions causing intracranial hypertension or hypotension while the pathogenesis of primary forms is still controversial.
There are several theories in the literature but the most accepted one implies an overcrowding due to a small posterior cranial fossa. While asymptomatic CIM do not need treatment, symptomatic ones prompt for surgical management. Several techniques are proposed being the dilemma centered in the need for dural opening procedures and bony decompression ones.
Alongside the paper, the authors will address the novelty presented in the literature on management, diagnosis and pathogenesis in order to offer a better understanding of such a heterogeneous pathology.
近年来，由于影像学评估的广泛应用，Chiari I 畸形已成为神经外科医生需要处理的主要疾病之一。根据小脑扁桃体尖端进入枕骨大孔的程度，可将 CIM 分为超过 5 毫米的突出，被认为是病理性的。这种疾病是一种具有多因素发病机制的异质性疾病，可以分为原发性和继发性。无论何种形式，似乎 CIM 都是由于颅腔容积与其内容物之间的失衡所致。获得性 CIM 继发于导致颅内压升高或降低的疾病，而原发性形式的发病机制仍存在争议。
文献中有几种理论，但最被接受的一种理论暗示由于后颅窝小而导致过度拥挤。无症状的 CIM 不需要治疗，而有症状的 CIM 需要手术治疗。提出了几种技术，其困境集中在需要硬脑膜开放术和骨减压术。
本文作者将讨论文献中关于管理、诊断和发病机制的新进展，以便更好地理解这种异质性病理。
