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

Jennifer Strahle, Karin M. Muraszko, Hugh J.L. Garton, Brandon W. Smith, Jordan Starr, Joseph R. Kapurch, Cormac O. Maher

Research output: Contribution to journalArticle

20 Scopus citations

Abstract

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 identifed 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.

Original languageEnglish
Pages (from-to)21-29
Number of pages9
JournalJournal of Neurosurgery: Pediatrics
Volume16
Issue number1
DOIs
StatePublished - Jul 2015
Externally publishedYes

Keywords

  • Chiari malformation
  • Congenital
  • Scoliosis
  • Syrinx

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    Strahle, J., Muraszko, K. M., Garton, H. J. L., Smith, B. W., Starr, J., Kapurch, J. R., & Maher, C. O. (2015). Syrinx location and size according to etiology: Identification of Chiari-associated syrinx. Journal of Neurosurgery: Pediatrics, 16(1), 21-29. https://doi.org/10.3171/2014.12.PEDS14463