title: Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia.
abstract: OBJECTIVE
  Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I-associated syringomyelia. A secondary objective was to explore the role of craniovertebral junction (CVJ) characteristics as additional risk factors for scoliosis.
  
  
  METHODS
  The authors conducted a retrospective review of pediatric patients evaluated for CM-I with syringomyelia at a single institution in the period from 2000 to 2012. Syrinx morphology and CVJ parameters were evaluated with MRI, whereas the presence of scoliosis was determined using standard radiographic criteria. Multiple logistic regression was used to analyze radiological features that were independently associated with scoliosis.
  
  
  RESULTS
  Ninety-two patients with CM-I and syringomyelia were identified. The mean age was 10.5 ± 5 years. Thirty-five (38%) of 92 patients had spine deformity; 23 (66%) of these 35 patients were referred primarily for deformity, and 12 (34%) were diagnosed with deformity during workup for other symptoms. Multiple regression analysis revealed maximum syrinx diameter > 6 mm (OR 12.1, 95% CI 3.63-40.57, p < 0.001) and moderate (5-12 mm) rather than severe (> 12 mm) tonsillar herniation (OR 7.64, 95% CI 2.3-25.31, p = 0.001) as significant predictors of spine deformity when controlling for age, sex, and syrinx location.
  
  
  CONCLUSIONS
  The current study further elucidates the association between CM-I and spinal deformity by defining specific radiographic characteristics associated with the presence of scoliosis. Specifically, patients presenting with larger maximum syrinx diameters (> 6 mm) have an increased risk of scoliosis.
authors: Departments of Orthopaedic Surgery and.
authors: Jakub
authors: J
authors: Godzik
authors: Michael P
authors: MP
authors: Kelly
authors: Alireza
authors: A
authors: Radmanesh
authors: David
authors: D
authors: Kim
authors: Terrence F
authors: TF
authors: Holekamp
authors: Matthew D
authors: MD
authors: Smyth
authors: Lawrence G
authors: LG
authors: Lenke
authors: Joshua S
authors: JS
authors: Shimony
authors: Tae Sung
authors: TS
authors: Park
authors: Jeffrey
authors: J
authors: Leonard
authors: David D
authors: DD
authors: Limbrick
mesh_terms: D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004677:Encephalocele / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D016015:Logistic Models; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence; D011859:Radiography; D012189:Retrospective Studies; D012307:Risk Factors; D012600:Scoliosis / Q000000981:diagnostic imaging* / Q000209:etiology*; D013595:Syringomyelia / Q000150:complications* / Q000473:pathology