Mancarella Cristina, Delfini Roberto, Landi Alessandro
Department of Neurology and Psychiatry, Division of Neurosurgery, "Sapienza" University of Rome, Rome, Italy.
Acta Neurochir Suppl. 2019;125:89-95. doi: 10.1007/978-3-319-62515-7_13.
Chiari malformations (CM) represent a group of anomalies characterized by descent of the cerebellar tonsils or vermis into the cervical spinal canal. These malformations can be associated with abnormalities such as hydrocephalus, spina bifida, hydromyelia, syringomyelia, curvature of the spine (kyphosis and scoliosis) and tethered cord syndrome. Hereditary syndromes and other disorders that affect growth and bone formation-such as craniosynostosis, Ehlers-Danlos syndromes and Klippel-Feil syndrome-can also be associated with CM.
The literature concerning treatment is large, and an extensive range of therapeutic protocols have been described. The literature is inclined in favour of surgery; however, there is controversy over when to perform surgery and which procedure is most appropriate. Lately, the indications for stabilization have been under discussion.
In this paper we review the literature and discuss the historical background, anatomical forms, pathophysiology, clinical presentation, relationships with other diseases and diagnostic procedures for these abnormalities.
Chiari畸形（CM）是一组以小脑扁桃体或蚓部下陷入颈椎管为特征的异常情况。这些畸形可能与脑积水、脊柱裂、脊髓积水、脊髓空洞症、脊柱弯曲（驼背和脊柱侧凸）以及脊髓栓系综合征等异常有关。遗传性综合征和其他影响生长及骨形成的疾病，如颅缝早闭、埃勒斯-当洛综合征和克利佩尔-费尔综合征，也可能与CM相关。
关于治疗的文献众多，已描述了广泛的治疗方案。文献倾向于手术治疗；然而，对于何时进行手术以及哪种手术最为合适存在争议。最近，关于稳定化治疗的指征一直在讨论中。
在本文中，我们回顾了文献，并讨论了这些异常情况的历史背景、解剖形式、病理生理学、临床表现、与其他疾病的关系以及诊断方法。
