Chiari-related scoliosis: a single-center experience with long-term radiographic follow-up and relationship to deformity correction

J Neurosurg Pediatr. 2018 Feb;21(2):185-189. doi: 10.3171/2017.8.PEDS17318. Epub 2017 Nov 24.

Abstract

OBJECTIVE Previous reports have addressed the short-term response of patients with Chiari-related scoliosis (CRS) to suboccipital decompression and duraplasty (SODD); however, the long-term behavior of the curve has not been well defined. The authors undertook a longitudinal study of a cohort of patients who underwent SODD for CRS to determine whether there are factors related to Chiari malformation (CM) that predict long-term scoliotic curve behavior and need for deformity correction. METHODS The authors retrospectively reviewed cases in which patients underwent SODD for CRS during a 14-year period at a single center. Clinical (age, sex, and associated disorders/syndromes) and radiographic (CM type, tonsillar descent, pBC2 line, clival-axial angle [CXA], syrinx length and level, and initial Cobb angle) information was evaluated to identify associations with the primary outcome: delayed thoracolumbar fusion for progressive scoliosis. RESULTS Twenty-eight patients were identified, but 4 were lost to follow-up and 1 underwent fusion within a year. Among the remaining 23 patients, 11 required fusion surgery at an average of 88.3 ± 15.4 months after SODD, including 7 (30%) who needed fusion more than 5 years after SODD. On univariate analysis, a lower CXA (131.5° ± 4.8° vs 146.5° ± 4.6°, p = 0.034), pBC2 > 9 mm (64% vs 25%, p = 0.06), and higher initial Cobb angle (35.1° ± 3.6° vs 22.8° ± 4.0°, p = 0.035) were associated with the need for thoracolumbar fusion. Multivariable modeling revealed that lower CXA was independently associated with a need for delayed thoracolumbar fusion (OR 1.12, p = 0.0128). CONCLUSIONS This investigation demonstrates the long-term outcome and natural history of CRS after SODD. The durability of the effect of SODD on CRS and curve behavior is poor, with late curve progression occurring in 30% of patients. Factors associated with CRS progression include an initial pBC2 > 9 mm, lower CXA, and higher Cobb angle. Lower CXA was an independent predictor of delayed thoracolumbar fusion. Further study is necessary on a larger cohort of patients to fully elucidate this relationship.

Keywords: CCJ = craniocervical junction; CM = Chiari malformation; CM-I = CM Type I; CRS = Chiari-related scoliosis; CXA = clival-axial angle; Chiari decompression; Cobb angle; SODD = suboccipital decompression and duraplasty; clival-axial angle; deformity correction; long-term follow-up; scoliosis; spine; suboccipital decompression and duraplasty; syringopleural shunt; syrinx; thoracolumbar fusion.

MeSH terms

  • Arnold-Chiari Malformation / complications
  • Arnold-Chiari Malformation / surgery*
  • Child
  • Decompression, Surgical / methods*
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Neurosurgical Procedures / methods*
  • Retrospective Studies
  • Scoliosis / etiology
  • Scoliosis / surgery*
  • Spinal Fusion / statistics & numerical data
  • Treatment Outcome