Chiari syndrome and respiratory failure: a literature review

J Intensive Care Med. 2014 Sep-Oct;29(5):260-8. doi: 10.1177/0885066613485213. Epub 2013 Apr 12.


Background: Patients with failed extubation requiring reintubation have increased morbidity and mortality. This situation may reflect the severity of the underlying disorder or may reflect an undiagnosed condition that was not apparent at the time of the initial intubation.

Methods: Case report and literature review.

Results: A 49-year-old man developed acute hypercapneic respiratory failure during an episode of community-acquired pneumonia. This patient failed extubation 4 times. Routine neurological evaluation, including computed tomography of the head, was within normal limits. A magnetic resonance imaging revealed a severe Chiari malformation type I. The patient eventually required tracheostomy and prolonged mechanical ventilation. We identified 28 cases with Chiari malformations (CM) in the medical literature, who presented with respiratory failure. The mean age was 29.5 years, 46% of the cases were male, 50% had an associated syringomyelia, and 64% required a decompressive surgery. Twenty-five percent had recurrent symptoms postsurgery.

Conclusions: Patients with CMs can have repeated extubation failures. Some of these patients have normal neurological examinations and studies and are not identified until they have an MRI study. Clinicians need to consider this possibility in patients who are difficult to wean.

Keywords: Chiari malformation; apnea; extubation; mechanical ventilation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Arnold-Chiari Malformation / complications*
  • Diagnostic Imaging
  • Humans
  • Intubation, Intratracheal
  • Male
  • Middle Aged
  • Respiration, Artificial
  • Respiratory Insufficiency / etiology*
  • Respiratory Insufficiency / therapy*
  • Tracheostomy