Natural history of Chiari malformation Type I following decision for conservative treatment

J Neurosurg Pediatr. 2011 Aug;8(2):214-21. doi: 10.3171/2011.5.PEDS1122.


Object: The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management.

Methods: The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis.

Results: Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not.

Conclusions: In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.

MeSH terms

  • Adolescent
  • Arnold-Chiari Malformation / pathology*
  • Arnold-Chiari Malformation / surgery
  • Arnold-Chiari Malformation / therapy*
  • Child
  • Child, Preschool
  • Disease Progression
  • Encephalocele / pathology
  • Encephalocele / surgery
  • Encephalocele / therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Imaging*
  • Male
  • Predictive Value of Tests
  • Retrospective Studies
  • Syringomyelia / pathology
  • Syringomyelia / surgery
  • Syringomyelia / therapy
  • Treatment Outcome