The Chiari I malformation

被引:92
作者
McClugage, Samuel G., III [1 ]
Oakes, W. Jerry [1 ]
机构
[1] Univ Alabama Birmingham, Dept Neurosurg, Birmingham, AL USA
关键词
Chiari I malformation; tonsillar ectopia; definition; diagnosis; treatment; POSTERIOR-FOSSA DECOMPRESSION; TONSILLAR HERNIATION; INCIDENTAL FINDINGS; DANDY-WALKER; SYRINGOMYELIA; MANAGEMENT; FORAMEN; ECTOPIA; EXPERIENCE; SCOLIOSIS;
D O I
10.3171/2019.5.PEDS18382
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:217 / 226
页数:10
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