Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management

World Neurosurg. 2017 Aug:104:213-219. doi: 10.1016/j.wneu.2017.04.082. Epub 2017 Apr 21.


Background: Chiari malformation type 1 (CM-1) is a variation of hindbrain development that can sometimes occur in asymptomatic individuals. Conventional treatment is surgical decompression, but little is known about the natural history of patients who do not undergo surgical management. This information is critical to determine how these patients should be managed. We conducted a systematic literature review to determine the natural history of CM-1, particularly in patients who did not undergo surgery and in asymptomatic individuals, to help patients and physicians determine when surgery is likely to be beneficial.

Methods: The literature search was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the electronic databases PubMed, Scopus, Cochrane Library, and Web of Science. Inclusion and exclusion criteria were predefined.

Results: In symptomatic patients who did not undergo surgery, headaches and nausea often improved, whereas ataxia and sensory disturbance tended not to improve spontaneously. Of patients, 27%-47% had an improvement in symptoms after 15 months, and 37%-40% with cough headache and 89% with nausea who were managed nonoperatively improved at follow-up. Most asymptomatic individuals with CM-1 remained asymptomatic (93.3%) even in the presence of syringomyelia.

Conclusions: The natural history of mild symptomatic and asymptomatic CM-1 in adults is relatively benign and nonprogressive; the decision to perform surgical decompression should be based on severity and duration of a patient's symptoms at presentation. It is reasonable to observe a patient with mild or asymptomatic symptoms even in the presence of significant tonsillar descent or syringomyelia.

Keywords: Adult; Arnold-Chiari; Chiari; Conservative; Malformation; Management; Surgical.

Publication types

  • Meta-Analysis
  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Arnold-Chiari Malformation / diagnosis*
  • Arnold-Chiari Malformation / epidemiology*
  • Arnold-Chiari Malformation / nursing
  • Asymptomatic Diseases / epidemiology
  • Ataxia / diagnosis
  • Ataxia / epidemiology*
  • Ataxia / prevention & control
  • Causality
  • Comorbidity
  • Disease Progression*
  • Evidence-Based Medicine
  • Female
  • Headache / diagnosis
  • Headache / epidemiology*
  • Headache / prevention & control
  • Humans
  • Incidence
  • Longitudinal Studies
  • Male
  • Nausea / diagnosis
  • Nausea / epidemiology*
  • Nausea / prevention & control
  • Risk Factors
  • Sensation Disorders / diagnosis
  • Sensation Disorders / epidemiology*
  • Sensation Disorders / prevention & control
  • Treatment Outcome