Chiari Type I Malformation Revisited: Diagnosis and Treatment

Abstract

BACKGROUND: Chiari type I malformations (Chiari I) are congenital deformities where caudal migration of the cerebellar tonsils through the foramen magnum compresses the cerebellum and cervicomedullary junction (lower brainstem and upper cervical spinal cord). Associations with chronic fatigue syndrome, fibromyalgia, orthostatic intolerance, and other neurologic syndromes have been proposed along with the current plethora of known symptoms of this disease process. In advanced cases, Chiari I malformations can lead to significant neurologic deficit and be the cause of permanent nervous system damage. REVIEW SUMMARY: This article focuses on the clinical diagnosis and treatment of patients with Chiari I, including a discussion on the possible mechanisms of Chiari I with a review of present diagnostic tests, indications for treatment, and appraisal of surgical outcome. CONCLUSIONS: Future radiological advances and research will undoubtedly be directed to better understanding of the pathology of the Chiari malformation and more effective medical and surgical treatment.