Syrinx location and size according to etiology: identification of Chiari-associated syrinx

J Neurosurg Pediatr. 2015 Jul;16(1):21-9. doi: 10.3171/2014.12.PEDS14463. Epub 2015 Apr 3.


OBJECT Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined. METHODS All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups. RESULTS A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I-associated and idiopathic syringes, the authors found that CM-I-associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92-0.99) for CM-I-associated syrinx. CONCLUSIONS Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I-associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.

Keywords: 2°CM = secondary CM; CM = Chiari malformation; CM-0 = CM Type 0; CM-I = CM Type I; Chiari malformation; congenital; scoliosis; syrinx.

MeSH terms

  • Adolescent
  • Arnold-Chiari Malformation / complications*
  • Arnold-Chiari Malformation / surgery
  • Child
  • Child, Preschool
  • Decompression, Surgical / adverse effects
  • Female
  • Humans
  • Male
  • Neural Tube Defects / complications
  • Scoliosis / complications
  • Spine / pathology*
  • Syringomyelia / etiology*
  • Syringomyelia / pathology*
  • Syringomyelia / surgery