McClugage Samuel G, Oakes W Jerry
J Neurosurg Pediatr. 2019 Sep 1;24(3):217-226. doi: 10.3171/2019.5.PEDS18382.
As with many pathologies, the course of our understanding of the Chiari I malformation (CIM) has developed extensively over time. The early descriptions of the Chiari malformations by Hans Chiari in 1891 opened the door for future classification and research on this topic. However, even over a long timeframe, our understanding of the pathophysiology and, more importantly, treatment, remained in its infancy. As recently as the 1970s, CIM was not discussed in popular neurology textbooks. Syringomyelia is listed as a degenerative disorder with no satisfactory treatment. Radiation therapy was considered an option in treatment, and surgery was thought to play no role. During the last 40 years, equivalent to the duration of a neurosurgical career, our understanding of the pathophysiology and natural history of CIM, coupled with modern MRI, has improved the treatment paradigm for this patient population. More importantly, it has given us evidence confirming that CIM is a disorder responsive to surgical intervention, giving patients once thought to be destined for lifelong disability a comparatively normal life after treatment. The purpose of this article is to offer a review of CIM and its important associated entities. The authors will discuss the evolution in understanding of the Chiari malformation and, importantly, distinguish between symptomatic CIM and asymptomatic tonsillar ectopia, based on imaging and presenting symptomatology. They will discuss techniques for surgical intervention, expected outcomes, and complications after surgery. Proper patient selection for surgery based on appropriate symptomatology is tantamount to achieving good surgical outcomes in this population, separating those who can be helped by surgery from those who are unlikely to improve. While our knowledge of the Chiari malformations continues to improve through the efforts of clinical and basic science researchers, surgeons, and patients, our current understanding of these entities represents a monumental improvement in patient care over a relatively short time period.
与许多疾病一样，随着时间的推移，我们对 Chiari I 型畸形（CIM）的认识有了广泛的发展。1891 年汉斯· Chiari 对 Chiari 畸形的早期描述为该主题的未来分类和研究打开了大门。然而，即使经过很长一段时间，我们对其病理生理学，更重要的是治疗的理解仍处于起步阶段。直到 20 世纪 70 年代，流行的神经病学教科书中都没有讨论 CIM。脊髓空洞症被列为一种退行性疾病，没有令人满意的治疗方法。放射治疗被认为是一种治疗选择，而手术被认为不起作用。在过去的 40 年里，相当于一个神经外科医生的职业生涯，我们对 CIM 的病理生理学和自然史的理解，再加上现代 MRI，改善了这一患者群体的治疗模式。更重要的是，它为我们提供了证据，证实 CIM 是一种对手术干预有反应的疾病，使曾经被认为注定要终身残疾的患者在治疗后过上相对正常的生活。本文的目的是对 CIM 及其重要的相关实体进行综述。作者将讨论对 Chiari 畸形认识的演变，重要的是，根据影像学和临床表现区分有症状的 CIM 和无症状的扁桃体异位。他们将讨论手术干预技术、预期结果和术后并发症。根据适当的症状对患者进行正确的手术选择对于在这一人群中取得良好的手术效果至关重要，将那些可以通过手术得到帮助的人与那些不太可能改善的人区分开来。虽然通过临床和基础科学研究人员、外科医生和患者的努力，我们对 Chiari 畸形的认识不断提高，但我们目前对这些实体的理解代表了在相对较短的时间内患者护理方面的巨大进步。
