Spinal Kyphoscoliosis Associated with Chiari Formation and Syringomyelia 'Recovery' Following Atlantoaxial Fixation: A Preliminary Report and Early Results Based on Experience with 11 Surgically Treated Cases

World Neurosurg. 2019 May:125:e937-e946. doi: 10.1016/j.wneu.2019.01.212. Epub 2019 Feb 11.

Abstract

Objective: Immediate postoperative and early clinical outcome of treatment of spinal kyphoscoliosis (SKS) associated with Chiari formation (CF) and syringomyelia (SM) and treated by atlantoaxial fixation is described.

Methods: During the period April 2016 to March 2018, 11 patients with SKS as a major presenting symptom in association with CF and SM were surgically treated and are analyzed retrospectively. The patients were 6 boys and 5 girls, and their ages ranged from 11-17 years (average 14 years). Six patients (54.5%) had associated neurologic symptoms and 9 patients had neck pain. All patients were treated by atlantoaxial fixation. No manipulation of foramen magnum bone or dura was performed. No surgery was performed on the subaxial cervical or dorsal spine. Patient Satisfaction Score was based on a specially designed questionnaire. Other outcome measures included clinical and radiologic assessment data, clinical photographs and video observation, and a Scoliosis Research Society Questionnaire.

Results: Apart from improvement in neurologic condition, more particularly in breathing capability and voice volume restoration, all patients and their relatives noticed recovery in SKS in the immediate postoperative phase. During the average follow-up of 14 months, all patients continued to have progressive symptomatic recovery and recovery from SKS. Patient Satisfaction Score suggested that all patients were 'highly' satisfied with the surgical outcome.

Conclusions: Atlantoaxial fixation in patients with CF and SM associated with SKS results in reversal of several musculoskeletal and neural abnormalities that includes recovery from spinal deformity.

Keywords: Atlantoaxial dislocation; Chiari formation; Kyphoscoliosis; Syringomyelia.

MeSH terms

  • Adolescent
  • Arnold-Chiari Malformation / complications*
  • Arnold-Chiari Malformation / surgery*
  • Atlanto-Axial Joint / surgery*
  • Child
  • Female
  • Humans
  • Kyphosis / etiology
  • Kyphosis / surgery*
  • Male
  • Scoliosis / etiology
  • Scoliosis / surgery
  • Spinal Fusion / methods*
  • Syringomyelia / complications*
  • Syringomyelia / surgery*
  • Treatment Outcome

Supplementary concepts

  • Chiari Malformation Type I with Syringomyelia