Morphometric and volumetric comparison of 102 children with symptomatic and asymptomatic Chiari malformation Type I

J Neurosurg Pediatr. 2018 Jan;21(1):65-71. doi: 10.3171/2017.8.PEDS17345. Epub 2017 Nov 10.

Abstract

OBJECTIVE Chiari malformation Type I (CM-I) is typically defined on imaging by a cerebellar tonsil position ≥ 5 mm below the foramen magnum. Low cerebellar tonsil position is a frequent incidental finding on brain or cervical spine imaging, even in asymptomatic individuals. Nonspecific symptoms (e.g., headache and neck pain) are common in those with low tonsil position as well as in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and nonspecific symptoms. Because cerebellar tonsil position is not strictly correlated with the presence of typical CM-I symptoms, the authors sought to determine if other 2D morphometric or 3D volumetric measurements on MRI could distinguish between patients with asymptomatic and symptomatic CM-I. METHODS The authors retrospectively analyzed records of 102 pediatric patients whose records were in the University of Michigan clinical CM-I database. All patients in this database had cerebellar tonsil position ≥ 5 mm below the foramen magnum. Fifty-one symptomatic and 51 asymptomatic patients were matched for age at diagnosis, sex, tonsil position, and tonsil morphology. National Institutes of Health ImageJ software was used to obtain six 2D anatomical MRI measurements, and a semiautomated segmentation tool was used to obtain four 3D volumetric measurements of the posterior fossa and CSF subvolumes on MRI. RESULTS No significant differences were observed between patients with symptomatic and asymptomatic CM-I related to tentorium length (50.3 vs 51.0 mm; p = 0.537), supraoccipital length (39.4 vs 42.6 mm; p = 0.055), clivus-tentorium distance (52.0 vs 52.1 mm; p = 0.964), clivus-torcula distance (81.5 vs 83.3 mm; p = 0.257), total posterior fossa volume (PFV; 183.4 vs 190.6 ml; p = 0.250), caudal PFV (152.5 vs 159.8 ml; p = 0.256), fourth ventricle volume to caudal PFV ratio (0.0140 vs 0.0136; p = 0.649), or CSF volume to caudal PFV ratio (0.071 vs 0.061; p = 0.138). CONCLUSIONS No clinically useful 2D or 3D measurements were identified that could reliably distinguish pediatric patients with symptoms attributable to CM-I from those with asymptomatic CM-I.

Keywords: CM-I = Chiari malformation Type I; Chiari malformation Type I; PFV = posterior fossa volume; morphometrics; syringomyelia; volumetrics.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Arnold-Chiari Malformation / pathology*
  • Brain Diseases / pathology*
  • Cerebellum / pathology
  • Child
  • Female
  • Foramen Magnum / pathology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Organ Size / physiology
  • Retrospective Studies