Scoliosis with Chiari I malformation without associated syringomyelia

Spine Deform. 2021 Jul;9(4):1105-1113. doi: 10.1007/s43390-021-00286-7. Epub 2021 Jan 20.

Abstract

Purpose: Many patients with presumed idiopathic scoliosis are found to have Chiari I malformation (CM-I) on MRI. The objective of this study is to report on scoliosis progression in CM-I with no syringomyelia.

Methods: A retrospective review of patients with scoliosis and CM-I was conducted from 1997 to 2015. Patients with syringomyelia and/or non-idiopathic scoliosis were excluded. Clinical and radiographic characteristics were recorded at presentation and latest follow-up. CM-I was defined as the cerebellar tonsil extending 5 mm or more below the foramen magnum on MRI.

Results: Thirty-two patients (72% female) with a mean age of 11 years (range 1-16) at scoliosis diagnosis were included. The average initial curve was 30.3° ± SD 16.3. The mean initial Chiari size was 9.6 mm SD ± 4.0. Fifteen (46.9%) experienced Chiari-related symptoms, and three (9%) patients underwent Posterior Fossa Decompression (PFD) to treat these symptoms. 10 (31%) patients went on to fusion, progressing on average 13.6° (95% CI 1.6-25.6°). No association was detected between decompression and either curve progression or fusion (p = 0.46, 0.60). For those who did not undergo fusion, curve magnitude progressed on average 1.0° (95% CI - 4.0 to 5.9°). There was no association between age, Chiari size, presence of symptoms, initial curve shape, or bracing treatment and fusion.

Conclusion: Patients with CM-I and scoliosis may not require surgical treatment, including PFD and fusion. Scoliosis curvature stabilized in the non-surgical population at an average progression of 1.0°. These results suggest that CM-I with no syringomyelia has minimal effect on scoliosis progression.

Keywords: Chiari I Malformation; Idiopathic Scoliosis; Posterior Fossa Decompression; Posterior Spinal Fusion.

MeSH terms

  • Adolescent
  • Arnold-Chiari Malformation* / complications
  • Arnold-Chiari Malformation* / diagnostic imaging
  • Arnold-Chiari Malformation* / surgery
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Male
  • Retrospective Studies
  • Scoliosis* / complications
  • Scoliosis* / diagnostic imaging
  • Scoliosis* / surgery
  • Syringomyelia* / complications
  • Syringomyelia* / diagnostic imaging
  • Syringomyelia* / surgery
  • Treatment Outcome