Arnold Chiari Malformation

Abstract

Chiari malformations (CM) refer to a series of anomalies characterized by the descent of cerebellar tonsils into the cervical spinal canal. These malformations can be associated with abnormalities such as syringomyelia, hydrocephalus, spina bifida, and scoliosis. Additionally, craniocervical junction anomalies, endocrinopathies, craniosynostosis, and syndromic disorders are also linked to CM. The treatment of CM is surgical, and there is no known medical therapy. Patients diagnosed with CM are typically advised to undergo surgical treatment or follow-up. Although surgical intervention is supported in the literature, debates exist regarding which procedure is most suitable and when surgery should be performed. In this article, we will examine the historical background of CM, its anatomical forms, pathophysiology, clinical presentation, relationship with other diseases, and diagnostic procedures in the light of the literature.